Left ventricular angiocardiography in atrioventricular defects.

SUMMARY A 64 year old woman with partial atrioventricular canal and double orifice mitral valve presented with severe mitral regurgitation secondary to a torn leaflet. The double orifice regurgitant mitral valve is an unusual finding at operation. Double orifice mitral valve is an unusual congenital abnormality that is rarely of clinical importance. We describe a patient in whom this finding was associated with severe mitral regurgitation.Case report A 64 year old woman was noted to have a murmur at age 20, but was symptom free until 1977 when congestive heart failure developed. A murmur typical of mitral regurgitation was noted. Her symptoms resolved on digoxin and diuretic treatment. She did well until November, 1983, when symptoms of weakness, abdominal distension, dyspnoea, and palpitation developed. Frusemide and quinidine (for ventricular extrasystoles) were started but when her symptoms progressed she was referred to our institution.-CLINICAL FINDINGS Physical examination showed a normotensive female in no distress. Pulse was 66 beats per minute with frequent extrasystoles. Her venous pressure was raised with pronounced V waves. Carotid pulses were normal. The apical impulse was felt in the sixth intercostal space in the midclavicular line, and there was a left parasternal thrill. The first and second heart sounds were normal. The third heart sound was soft. A grade 5/6 harsh pansystolic murmur radiated from the heart apex to the axilla and spine. The electrocardiogram showed left axis deviation, first degree atrioventricular block, and right bundle branch block. A chest x ray film showed cardiomegaly, prominent pulmonary arteries, and increased pulmonary vascular flow. Laboratory studies were unremarkable.Cross sectional echocardiography (Fig. 1) showed left atrial, right atrial, and right ventricular enlargement; an ostium primum type atrial septal defect; and a cleft anterior mitral leaflet. A contrast study demonstrated right to left shunting across the atrial septal defect. At retrospective examination of the echocardiogram two separate mitral valve orifices could be seen in the parasternal long axis right ventricular inflow view.Cardiac catheterisation demonstrated raised right heart pressures, increased right atrial oxygen saturation and mild arterial desaturation. The pulmonary: systemic blood flow ratio was 1 9:1.Left ventricular cineangiography showed normal wall motion with evidence of a gooseneck deformity of the left ventricular outflow tract and severe mitral regurgitation. There was left atrial and right ventricular enlargement. Early appearance of contrast in the right atrium confirmed the presence of an atrial septal defect. Coronary angiography was normal. FINDINGS AT OPERATIONThe heart was explored through a lateral right atrial incision. The tricuspid valve annulus was considerably dilated with a cleft in the septal leaflet extending to the septal rim. The valve was grossly incompetent. A 3 cm diameter ostium primum defect was present. The mitral valve was fully viewed through an incis...

Section: Discussionmentioning

confidence: 99%

Section: Angiographic and Echocardiographic Findingsmentioning

confidence: 99%

Section: Surgical Considerationsmentioning

confidence: 99%

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Severe mitral regurgitation in a woman with a double orifice mitral valve.

Kron¹,

Standerfer²,

Starr³

1986

“…Some have suggested that all the membranous portion of the ventricular septum is lacking (Rogers and Edwards, 1948;Wakai and Edwards, 1956;Campbell and Missen, 1957;Wakai and Edwards, 1958;Neufeld et al, 1961). Others have suggested that the atrioventricular membranous septum together with the basal portion of the muscular ventricular septum are missing (Van Mierop et al, 1962;Al Omeri et al 1965;Somerville, 1966;Van Mierop and Alley, 1966;Somerville and Jefferson, 1968). Yet others pointed to a deficiency in the muscular rim beneath the membranous septum which extended to the diaphragmatic wall of the left ventricle (Goor et al, 1968) or to the subaortic area (Blieden et al, 1974).…”

Section: Discussionmentioning

confidence: 99%

Morphology and classification of atrioventricular defects.

Piccoli¹,

Gerlis²,

Wilkinson³

et al. 1979

102

Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.

“…The angiograms were examined by the authors and radiologists separately, and the anatomy of the mitral valve assessed as in previous studies (Criley et al, 1966;Grossman et al, 1968). Anteroposterior and lateral views were chosen in this study as they are the best for distinguishing atrioventricular defects from simpler forms of atrial septal defect (Somerville and Jefferson, 1968); in the first 10 patients this was the main clinical differential diagnosis. The same technique was used electively in the other patients in order to examine the diagnostic accuracy of the investigation.…”

Section: Subjectsmentioning

confidence: 99%

Prolapsed mitral cusps in atrial septal defect. An erroneous radiological interpretation.

Somerville¹,

Kaku²,

Saravalli³

1978

Self Cite

The association of ballooning of the cusps of the mitral valve with atrial septal defect has been documented from angiographic studies (McDonald et al., 1971;Pocock and Barlow, 1971;Epstein and Coulshed, 1973;McKay and Yacoub, 1973;Schwartz et al., 1975). The patient with an atrial septal defect who has left ventriculography is often the one in whom a mitral valve abnormality is suspected, either because of suggestive clinical signs or because of unexplained left axis deviation on the electrocardiogram. Thus, there has been the temptation to assume that the unusual appearance of the mitral valve seen on the angiocardiogram represents a pathological abnormality. In order to test the accuracy of left ventriculography in diagnosing prolapsed mitral cusps in simple atrial septal defect, the angiocardiographic images have been correlated with the anatomy of the mitral valve carefully examined at open operation by experienced surgeons. SubjectsForty patients with simple septal defect had left ventriculography before open heart surgery. Patients were aged 4 to 64 years at the time of 'Present address: