Editorial Type: Case Reports
 | 
Online Publication Date: 01 Nov 2005

Quadricuspid Aortic Valve and Associated Abnormalities in a Dog

BVSc,
DVM, Diplomate ACVIM (Cardiology), and
MMedVet (Radiology), Diplomate ECVDI
Article Category: Other
Page Range: 406 – 412
DOI: 10.5326/0410406
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An 11-month-old, female Scottish terrier was presented with a history of a heart murmur. The electrocardiogram showed signs of left ventricular enlargement, and radiography confirmed generalized cardiomegaly. Echocardiography revealed four equally sized aortic valve cusps. A ventricular septal defect, with systolic left-to-right shunting, and aortic regurgitation into both ventricles were also present. The dog was free of clinical signs 1 year after diagnosis.

Introduction

Quadricuspid aortic valve is a rare, congenital defect in which four valve cusps are present, rather than the usual three equally sized ones.1–5 Abnormal valve cusp numbers (either increased or decreased) are described for both humans and animals in the pulmonic valve, the aortic valve, and the truncus arteriosus.1,3,4,6–8 Uni-, bi-, and pentcuspid aortic valves exist, with bicuspid valves being the most common form.1,2 In humans, a quadricuspid aortic valve often is clinically insignificant and is frequently diagnosed incidentally on postmortem examination, during aortic valvular surgery, or later in life, from progressive aortic regurgitation and clinical deterioration.5,9,10 Although well described in the human literature, there is little written about this condition in the mainstream English veterinary literature.9 In animals, quadricuspid aortic valve has been described in the dog, cat, Syrian hamster, and the greater white-toothed shrew.3,4,6,11 The purposes of this report are to further describe and highlight this rare cardiac condition.

Case Report

An 11-month-old, 8.3-kg, female Scottish terrier in good health and body condition and with no prior history of illness, was referred to the Onderstepoort Veterinary Academic Hospital, University of Pretoria, for a cardiac evaluation before ovariohysterectomy. The referring veterinarian had detected a heart murmur on routine clinical examination. Clinical examination revealed pink mucous membranes and a capillary refill time of 1.5 seconds. Body temperature was 39.2°C, and heart rate was 112 beats per minute. A grade 6/6 holosystolic murmur was present over the right apex, with a 2/6 diastolic murmur heard loudest over the left heart base [Figures 1A, 1B]. The rhythm was regular, and the pulses were hyperdynamic (i.e., bounding). There were no other significant clinical or hematological findings. Electrocardiography (ECG) demonstrated a normal sinus rhythm and tall R waves, suggestive of left ventricular hypertrophy.

Standard right lateral and dorsoventral thoracic radiographs showed generalized cardiomegaly (vertebral heart scale of 11.5; dorsal elevation of trachea on the lateral view). Loss of the cranial cardiac waist on the lateral view and cranial mediastinal widening on the dorsoventral view suggested enlargement of the aortic root. The pulmonary parenchyma showed no significant abnormalities.

Two-dimensional, spectral, and color-flow Doppler echocardiography was performed several times over a 6-month period using a 5- to 6-MHz variable-phased array transducer.a Left and right lateral recumbent-dependent imaging was used. On occasion, sedation with intramuscular butorphanolb (1.0 mg) and acepromazinec (0.1 mg) was administered to aid the imaging. A right parasternal short-axis view of the aortic valve in diastole demonstrated four equally sized cusps, indicative of a quadricuspid aortic valve [Figure 2]. During systole, the valve was fully opened with no evidence of adhesions, thickenings, or vegetations. Identification of the type or origin of the extra cusp was not possible because of the symmetry of the cusps. The aortic valve annulus appeared normal for only about half its circumference; the segment surrounding the two cusps adjacent to, and protruding into, the right ventricle appeared less echogenic and thinner [Figure 2]. In the right long-axis four-chamber view [Figure 3], the aortic root protruded into the right ventricle. Evaluation of the basal part of the inter-ventricular septum demonstrated a perimembranous ventricular septal defect (VSD) [Figures 4A–4C]. A restrictive physiology was evidenced by a left-to-right velocity gradient of 5 meters per second across the defect. Increased left atrial pressure was suspected because of bowing of the interatrial septum into the right atrium.

M-mode evaluation [see Table] revealed dilatation of the left ventricle, with normal fractional shortening. Doppler cardiac evaluation [see Table] showed a normal to increased laminar systolic pulmonary artery velocity profile and no retrograde diastolic flow, ruling out a patent ductus arteriosus. There was no evidence of pulmonary valvular regurgitation. Systolic aortic velocity was normal. Aortic insufficiency [Figure 5A] was evident, with the diastolic flow partially directed into the right ventricle via the VSD. Additionally, diastolic flow in the right ventricular outflow tract (RVOT) was seen in the short-axis view at the level of the sinuses of Valsalva [Figure 5B]. On color Doppler interrogation, the flow appeared to be originating from the aortic root via a 2-mm connection [Figure 5B, arrowhead]. Minimal mitral valve regurgitation was detected. Laminar mitral-peak E-wave velocity was slightly increased, most likely from the left-to-right shunting.

Contrast echocardiography (i.e., agitated saline rapidly injected into a cephalic vein) was performed on two occasions and failed to identify a positive contrast effect (i.e., right-to-left shunting). A clear negative contrast effect (i.e., left-to-right shunting) was not observed. Aerobic and anaerobic bacterial blood cultures were negative. The clinical diagnoses were congenital quadricuspid aortic valve, left-to-right shunting, restrictive subcristal perimembranous VSD, and aortic regurgitation. Ovariohysterectomy, performed under sedation with epidural anesthesia, was uneventful.

During the subsequent 6-month period, ECG findings remained unchanged. Radiographically, the vertebral heart scale increased to 12.5 [Figures 6A, 6B]. The other previous findings remained unchanged. Repeat M-mode measurements indicated worsening of the volume overload. There was also a generalized increase in septal thickness and an increase in left ventricular diameter. Fractional shortening remained within normal limits. Overtly, the dog remained active, had no physical manifestations of cardiac decompensation, and required no treatment. Telephone follow-up with the owner 1 year later indicated no deterioration in the dog’s condition.

Discussion

Clinically, a congenital quadricuspid aortic valve must be differentiated from an acquired pseudoquadricuspid aortic valve that results from pathological processes such as inflammatory valvular disease and bacterial endocarditis.9 This dog was diagnosed with congenital quadricuspid aortic valve on the basis of age, the echocardiographic appearance of the valve leaflets (i.e., no evidence of thickening, vegetations, malformations, or calcification), negative blood cultures, and the absence of systemic clinical abnormalities (e.g., fever, septicemia, inflammatory leukogram). The quadricuspid aortic valve protruded into the right ventricle in association with a VSD, but the protrusion was clinically insignificant. No systemic signs of right-to-left shunting were noted, and an echocardiographic contrast study did not reveal contrast agent in the ascending aorta. A small proportion of aortic regurgitation was deviated across the VSD in the right ventricle [Figure 5A], but the right ventricle was not appreciably enlarged from its share of aortic regurgitation. There was evidence of diastolic collapse of the quadricuspid aortic valve [Figure 3], as has been found in association with VSD and aortic regurgitation in dogs with normally formed aortic valves.12 Other findings, such as left ventricular volume overload and resultant accelerated flow through the great vessels, were interpreted as changes secondary to aortic regurgitation and the VSD. An apparent communication between the aorta and RVOT [Figure 5B, arrowhead] may have arisen from an echocardiographic anomaly, a coronary artery, or an undefined flow, or it may have represented a true anatomical connection between the two structures. Further diagnostic evaluations, such as selective angiography, to clarify this issue were declined by the owner.

Quadricuspid aortic valve is predominantly an isolated congenital cardiac deformity.13–15 In humans, the incidence varies between 0.003% and 0.033% on postmortem examinations and between 0% and 0.3% for all aortic valve surgeries.1,2,10,14–16 The incidence rates have been 0.3% to 2.4% in cases of surgically replaced regurgitant aortic valves and 0.013% to 0.043% on echocardiographic examination.5,10,17,18 In people, quadricuspid pulmonic valves are five to 10 times more common than their aortic counterparts.1,13,14,19 Quadricuspid aortic valves may cause no clinical signs in humans, despite common functional abnormalities such as regurgitation or rare stenosis.9,14,16,18 Aortic regurgitation occurs in 43% to 56% of humans with quadricuspid aortic valve and was present in two dogs previously described with the condition.3,9,13 Subjectively, the degree of aortic regurgitation in this dog was substantially greater than reported earlier, but the division of the aortic regurgitation jet into right and left ventricular segments prevented its quantification in the present case.3 No concurrent cardiac defects were reported in two canine cases, but the dog reported here had a VSD and protrusion of the aortic valve into the right ventricle.3 In humans, quadricuspid aortic valves have been associated with numerous other cardiac abnormalities, including VSD, aortic regurgitation, sinus of Valsalva aneurysms, and aortic to right ventricular fistula.5,20–26 Quadricuspid aortic valve may also be associated with coronary artery abnormalities.10,11,20 In this dog, no coronary abnormalities were identified on echocardiography, similar to a previous report.3

Morphogenesis of quadricuspid aortic valve has been described in the Syrian hamster.6,27,28 The formation of abnormal pulmonic and aortic valves relies on two morphogenetic mechanisms, namely fusion and partitioning of valve cushions.6,28,29 Abnormalities in the two valves appear to occur independently, suggesting that the malformations have unique origins.6,28,29 Normally there are three valve cushions consisting of a mesenchymal core and endothelium cover, which develop into the normal three-cusped aortic valve.27 During quadricuspid aortic valve morphogenesis, globoidal endothelial cells have been found on the luminal side of the left aortic valve cushion, invaginating into it, and forming a recess.6 A single valve cushion, therefore, appears to split into two, resulting in the development of four valve leaflets. The genetic basis of a quadricuspid aortic valve is not yet fully understood, but it has been described in a case of a dominant hereditary malformation syndrome in one human family.30

Seven anatomical cusp variations of quadricuspid aortic valves have been described, and these may consist of a combination of equally or unequally sized cusps.14 The accessory cusp’s position may lie between any of the other cusps.21 Small or large accessory cusps increase the likelihood of aortic regurgitation because of an unequal distribution of stress on the cusps, while equally sized cusps are less likely to result in aortic regurgitation.5,31 In two dogs with quadricuspid aortic valves, one dog had equally sized cusps, while the other dog had two cusps of equal size and two that were substantially smaller.3 Both dogs had mild aortic regurgitation.3

Ventricular septal defects may cause aortic insufficiency, which results from loss of aortic root support at the septal lesion.12 Collapse of the aortic root and sinus of Valsalva into the septal defect can cause poor valve coaptation and secondary valve leaflet changes (e.g., thickening and rolling) from traumatic turbulent blood flow, which may further compromise leaflet function.12 As quadricuspid aortic valves regurgitate in only about half of affected humans, the aortic regurgitation in this case may be secondary to either the VSD or the quadricuspid valve morphology alone, or a combination of both.3,9,13 In this case, valve annulus collapse was likely to be accentuated because of the less echogenic, thinner, aortic valve annulus.

Congenital quadricuspid aortic valves typically have no evidence of inflammatory changes; but fenestration, calcification, and thickening of cusps may be seen.32–34 The commissurae between cusps may be slightly or completely fused, and a raphe-like ridge extending from the valve cusp to the vessel wall may exist, separating the cusp sinus into two cavities.25,28 Myxoid degeneration, fibrosis, and hyalinization may also be seen.15,16,18,32,35 No histological description exists of the one canine quadricuspid aortic valve examined on postmortem.3

Conclusion

In the case presented, the predominant findings included a quadricuspid aortic valve, VSD, and aortic regurgitation into both ventricles. This case was different from the two dogs in a previous report, in that concurrent cardiac abnormalities were found in association with a quadricuspid aortic valve.3 Despite the gross echocardiographic and radiological abnormalities noted, the dog remained free of clinical signs for the 1 year it was followed after diagnosis. The long-term prognosis remained guarded, however, because of the irreversibility of the primary lesions and presence of secondary changes.

a

Siemens Sonoline Omnia, Ehrlangen, Germany

b

Torbugesic; Fort Dodge Animal Health, Fort Dodge, IA 50501

c

Aceprom 2; Bayer, Isando, Republic South Africa

Table M-Mode Echocardiographic and Spectral Doppler Findings in a Dog With a Quadricuspid Aortic Valve
Table
Figures 1A, 1B—. Phonocardiogram recording of an 11-month-old Scottish terrier, done over the left base (A) and right apex (B) with simultaneous lead II electrocardiogram (25 mm/sec, 1 cm=1 mV) demonstrating a holosystolic murmur (S) and a less intense decrescendo diastolic murmur (D).Figures 1A, 1B—. Phonocardiogram recording of an 11-month-old Scottish terrier, done over the left base (A) and right apex (B) with simultaneous lead II electrocardiogram (25 mm/sec, 1 cm=1 mV) demonstrating a holosystolic murmur (S) and a less intense decrescendo diastolic murmur (D).Figures 1A, 1B—. Phonocardiogram recording of an 11-month-old Scottish terrier, done over the left base (A) and right apex (B) with simultaneous lead II electrocardiogram (25 mm/sec, 1 cm=1 mV) demonstrating a holosystolic murmur (S) and a less intense decrescendo diastolic murmur (D).
Figures 1A, 1B — Phonocardiogram recording of an 11-month-old Scottish terrier, done over the left base (A) and right apex (B) with simultaneous lead II electrocardiogram (25 mm/sec, 1 cm=1 mV) demonstrating a holosystolic murmur (S) and a less intense decrescendo diastolic murmur (D).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Figure 2—. Two-dimensional echocardiogram of the right parasternal short-axis view at the level of the aortic valve during diastole of the dog in Figure 1. Four equally sized cusps (marked with asterisks) can be seen. Diastolic coaptation of the four valve leaflets can be seen as an “X” in the center of the valve. Arrows indicate two aortic valve cusps adjacent to the right ventricle and overriding the interventricular septum. The base of these cusps was less echogenic and appeared thinner, suggesting hypoplasia (PA=pulmonary artery).Figure 2—. Two-dimensional echocardiogram of the right parasternal short-axis view at the level of the aortic valve during diastole of the dog in Figure 1. Four equally sized cusps (marked with asterisks) can be seen. Diastolic coaptation of the four valve leaflets can be seen as an “X” in the center of the valve. Arrows indicate two aortic valve cusps adjacent to the right ventricle and overriding the interventricular septum. The base of these cusps was less echogenic and appeared thinner, suggesting hypoplasia (PA=pulmonary artery).Figure 2—. Two-dimensional echocardiogram of the right parasternal short-axis view at the level of the aortic valve during diastole of the dog in Figure 1. Four equally sized cusps (marked with asterisks) can be seen. Diastolic coaptation of the four valve leaflets can be seen as an “X” in the center of the valve. Arrows indicate two aortic valve cusps adjacent to the right ventricle and overriding the interventricular septum. The base of these cusps was less echogenic and appeared thinner, suggesting hypoplasia (PA=pulmonary artery).
Figure 2 — Two-dimensional echocardiogram of the right parasternal short-axis view at the level of the aortic valve during diastole of the dog in Figure 1. Four equally sized cusps (marked with asterisks) can be seen. Diastolic coaptation of the four valve leaflets can be seen as an “X” in the center of the valve. Arrows indicate two aortic valve cusps adjacent to the right ventricle and overriding the interventricular septum. The base of these cusps was less echogenic and appeared thinner, suggesting hypoplasia (PA=pulmonary artery).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Figure 3—. Two-dimensional echocardiogram of the right parasternal long-axis, four-chamber view in diastole of the dog in Figure 2. The aortic valve cannot be identified as quadricuspid in the long-axis view. The sinus of Valsalva (SV) is protruding into the right ventricle (av=aortic valve leaflets, AoR=aortic root, ivs=interventricular septum, LV=left ventricle, RV=right ventricle).Figure 3—. Two-dimensional echocardiogram of the right parasternal long-axis, four-chamber view in diastole of the dog in Figure 2. The aortic valve cannot be identified as quadricuspid in the long-axis view. The sinus of Valsalva (SV) is protruding into the right ventricle (av=aortic valve leaflets, AoR=aortic root, ivs=interventricular septum, LV=left ventricle, RV=right ventricle).Figure 3—. Two-dimensional echocardiogram of the right parasternal long-axis, four-chamber view in diastole of the dog in Figure 2. The aortic valve cannot be identified as quadricuspid in the long-axis view. The sinus of Valsalva (SV) is protruding into the right ventricle (av=aortic valve leaflets, AoR=aortic root, ivs=interventricular septum, LV=left ventricle, RV=right ventricle).
Figure 3 — Two-dimensional echocardiogram of the right parasternal long-axis, four-chamber view in diastole of the dog in Figure 2. The aortic valve cannot be identified as quadricuspid in the long-axis view. The sinus of Valsalva (SV) is protruding into the right ventricle (av=aortic valve leaflets, AoR=aortic root, ivs=interventricular septum, LV=left ventricle, RV=right ventricle).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).
Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).
Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).Figures 4A–4C—. (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).
Figures 4A–4C — (A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).
Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).Figures 5A, 5B—. Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).
Figures 5A, 5B — Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).
Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).Figures 6A, 6B—. (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).
Figures 6A, 6B — (A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).

Citation: Journal of the American Animal Hospital Association 41, 6; 10.5326/0410406

Footnotes

    Doctor Côté’s current address is the Department of Companion Animals, Atlantic Veterinary College, University of Prince Edward Island, Charlottetown, Prince Edward Island, PE C1A 4P3, Canada.

References

  • 1
    Simonds JP. Congenital malformation of the aortic and pulmonary valves. Am J Med Sci 1923;166:584–595.
  • 2
    Edwards WD. Quadricuspid aortic valves. Am J Cardiovasc Pathol 1990;3:185.
  • 3
    Sisson D, Riepe R. Congenital quadricuspid aortic valve anomaly in two dogs. J Vet Cardiol 2000;2:25–28.
  • 4
    Harpster NK. Cardiovascular disease of the domestic cat. Adv Vet Sci Comp Med 1977;21:39–74.
  • 5
    Feldman BJ, Khandheria BK, Warnes CA, et al. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol 1990;65:937–938.
  • 6
    Fernandez B, Durán AC, Martire A, et al. New embryological evidence for the formation of quadricuspid aortic valves in the Syrian hamster (Mesocricetus auratus). J Comp Pathol 1999;121:89–94.
  • 7
    de la Cruz MV, Cayre R, Angelini P, et al. Coronary arteries in truncus arteriosus. Am J Cardiol 1990;66:1482–1486.
  • 8
    Van M, Patterson DF, Schnarr WR. Pathogenesis of persistent truncus arteriosus in light of observations made in a dog embryo with the anomaly. Am J Cardiol 1978;41:755–762.
  • 9
    Janssens U, Klues HG, Hanrath P. Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature. Heart 1997;78:83–87.
  • 10
    Bortolotti U, Scioti G, Levantino M, et al. Aortic valve replacement for quadricuspid aortic valve incompetence. J Heart Valve Dis 1998;7:515–517.
  • 11
    Durán AC. Quadricuspid aortic valve and single coronary artery in a greater white-toothed shrew, Crocidura russula. J Wildlife Dis 1996;32:658–660.
  • 12
    Sisson D, Luethy M, Thomas WP. Ventricular septal defect accompanied by aortic regurgitation in five dogs. J Am Anim Hosp Assoc 1991;27:441–448.
  • 13
    Davia JE, Fenoglio JJ, DeCastro CM, et al. Quadricuspid semilunar valves. Chest 1977;72:186–189.
  • 14
    Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623–626.
  • 15
    Tayama E, Chihara S, Kosuga T, et al. A case report of surgical treatment of quadricuspid aortic valve associated with regurgitation. Ann Thorac Cardiovasc Surg 2000;6:130–133.
  • 16
    Subramanian R, Olson LJ, Edwards WD. Surgical pathology of combined aortic stenosis and insufficiency: a study of 213 cases. Mayo Clin Proc 1985;60:247–254.
  • 17
    Waller BF, Taliercio CP, Dickos DK, et al. Rare or unusual causes of chronic, isolated, pure aortic regurgitation. Clin Cardiol 1990;13: 577–581.
  • 18
    Olson LJ, Subramanian R, Edwards WD. Surgical pathology of pure aortic insufficiency: a study of 225 cases. Mayo Clin Proc 1984; 59:835–841.
  • 19
    Bogers AJ, Bartelings MM, Bokenkamp R, et al. Common arterial trunk, uncommon coronary arterial anatomy. J Thorac Cardiovasc Surg 1993;106:1133–1137.
  • 20
    Rosenkranz ER, Murphy DJJ, Cosgrove DM. Surgical management of left coronary artery ostial atresia and supravalvar aortic stenosis. Ann Thorac Surg 1992;54:779–781.
  • 21
    Fernicola DJ, Mann JM, Roberts WC. Congenitally quadricuspid aortic valve: analysis of six necropsy patients. Am J Cardiol 1989;63:136–138.
  • 22
    Unger P, Preumont N, Stoupel E. Images in cardiology. Ruptured sinus of Valsalva aneurysm with right ventricular obstruction, quadricuspid aortic valve, and ventricular septal defect. Heart 2000;84:424.
  • 23
    Kurosawa H, Wagenaar SS, Becker AE. Sudden death in a youth. A case of quadricuspid aortic valve with isolation of origin of left coronary artery. Br Heart J 1981;46:211–215.
  • 24
    Moreno R, Zamorano J, De M, et al. Congenital quadricuspid aortic valve associated with congenital complete heart block. Eur J Echocardiogr 2002;3:236–237.
  • 25
    Fischler D, Fitzmaurice M, Ratliff NBJ. Quadricuspid aortic valve. Am J Cardiovasc Pathol 1990;3:91–94.
  • 26
    Chandrasekaran K, Tajik AJ, Edwards WD, et al. Two-dimensional echocardiographic diagnosis of quadricuspid aortic valve. Am J Cardiol 1984;53:1732–1733.
  • 27
    Fernandez B, Duran AC, Thiene G, et al. Embryological evidence for the formation of a quadricuspid aortic valve in the Syrian hamster. Cardiovasc Pathol 1994;3:287–291.
  • 28
    Fernandez B, Fernandez MC, Duran AC, et al. Anatomy and formation of congenital bicuspid and quadricuspid pulmonary valves in Syrian hamsters. Anat Rec 1998;250:70–79.
  • 29
    Sans-Coma V, Cardo M, Thiene G, et al. Bicuspid aortic and pulmonary valves in the Syrian hamster. Int J Cardiol 1992;34:249–254.
  • 30
    Kantaputra PN, Yamasaki K, Ishida T, et al. A dominantly inherited malformation syndrome with short stature, upper limb anomaly, minor craniofacial anomalies, and absence of TBX5 mutations: report of a Thai family. Am J Med Genet 2002;111:301–306.
  • 31
    Mathison M, Furuse A, Kotsuda H, et al. Quadricuspid aortic valve: case reports. Kyobu Geka 1990;43:383–388.
  • 32
    Bonde P, Sachithanandan A, McClements B, et al. Quadricuspid aortic valve: a rare cause of aortic insufficiency. J Heart Valve Dis 2002;11:506–508.
  • 33
    Wyatt JP, Goldenberg H. Supernumerary aortic cusps with multiple fenestrations and with displacement of the left coronary orifice. Arch Pathol Lab Med 1948;45:784–786.
  • 34
    Aoyagi S, Kawara T, Yasunaga H, et al. Congenital quadricuspid aortic valve associated with aortic regurgitation. Thorac Cardiovasc Surg 1992;40:225–226.
  • 35
    James KB, Centorbi LK, Novoa R. Quadricuspid aortic valve. Tex Heart Inst J 1991;18:141–143.
Copyright: Copyright 2005 by The American Animal Hospital Association 2005
<bold> <italic toggle="yes">Figures 1A, 1B</italic> </bold> —
Figures 1A, 1B —

Phonocardiogram recording of an 11-month-old Scottish terrier, done over the left base (A) and right apex (B) with simultaneous lead II electrocardiogram (25 mm/sec, 1 cm=1 mV) demonstrating a holosystolic murmur (S) and a less intense decrescendo diastolic murmur (D).

<bold> <italic toggle="yes">Figure 2</italic> </bold> —
Figure 2 —

Two-dimensional echocardiogram of the right parasternal short-axis view at the level of the aortic valve during diastole of the dog in Figure 1. Four equally sized cusps (marked with asterisks) can be seen. Diastolic coaptation of the four valve leaflets can be seen as an “X” in the center of the valve. Arrows indicate two aortic valve cusps adjacent to the right ventricle and overriding the interventricular septum. The base of these cusps was less echogenic and appeared thinner, suggesting hypoplasia (PA=pulmonary artery).

<bold> <italic toggle="yes">Figure 3</italic> </bold> —
Figure 3 —

Two-dimensional echocardiogram of the right parasternal long-axis, four-chamber view in diastole of the dog in Figure 2. The aortic valve cannot be identified as quadricuspid in the long-axis view. The sinus of Valsalva (SV) is protruding into the right ventricle (av=aortic valve leaflets, AoR=aortic root, ivs=interventricular septum, LV=left ventricle, RV=right ventricle).

<bold> <italic toggle="yes">Figures 4A–4C</italic> </bold> —
Figures 4A–4C —

(A) Two-dimensional echocardiogram of the right parasternal long-axis, left ventricular outflow view during systole. The ventricular septal defect (VSD) lies just apical to the aortic valve, which is protruding into the right ventricle (RV). (B) Color Doppler echocardiogram during systole, demonstrating turbulent flow into the RV through the VSD. (C) Continuous-wave Doppler imaging demonstrating systolic left-to-right flow through the VSD and diastolic aortic regurgitation (AR=aortic regurgitation, ivs=interventricular septum, LV=left ventricle, SV=sinus of Valsalva).

<bold> <italic toggle="yes">Figures 5A, 5B</italic> </bold> —
Figures 5A, 5B —

Two-dimensional echocardiogram with color-flow Doppler. (A) Right parasternal long-axis, left ventricular outflow view demonstrating aortic regurgitation. Some of the regurgitation is shunted left-to-right via the ventricular septal defect (arrow). (B) Right parasternal short-axis view during diastole at the level of the aortic valve. The arrowhead points to a color signal that could represent flow in a coronary artery, flow through an aortic-to-right-ventricular communication, or an artifact (AR=aortic regurgitation, LV=left ventricle, AV=aortic valve, PV=pulmonary valve, RVOT=right ventricular outflow tract, RV=right ventricle).

<bold> <italic toggle="yes">Figures 6A, 6B</italic> </bold> —
Figures 6A, 6B —

(A) Right lateral and (B) dorsoventral radiographs of the thorax taken 6 months after presentation. The vertebral heart scale score is 12.5, and there is elevation of the thoracic trachea indicative of cardiomegaly. A loss of the cranial cardiac waist is consistent with mild enlargement of the aortic root (L=left).

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