Br Heart J: first published as 10.1136/hrt.29.1.83 on 1 January 1967. Downloaded from Brit. Heart J., 1967, 29, 83. Congenital Mitral Stenosis S. P. SINGH*, M. S. GOTSMAN, L. D. ABRAMS, R. ASTLEY, C. G. PARSONS, AND K. D. ROBERTS From the Heart Unit, Birmingham Children's Hospital We have studied 8 children with congenital mitral more obvious. Signs of mitral stenosis persisted and stenosis and a left ventricle of approximately normal the pulmonary arterial pressure remained high (90/50 size. Ofthese, 4 are known to have had endocardial mm. Hg, in 1959). Eventually, at the age of 13, atrial fibro-elastosis, 2 had a persistent ductus arteriosus, fibrillation and cardiac failure developed and response to aortic and 1 had an abnormal medical treatment was unsatisfactory. A second valvo- 1 had stenosis, inferior tomy using cardiopulmonary bypass was attempted in vena cava. The presenting symptoms were re- 1959. The re-stenosis was confirmed, but unfortunately current respiratory infections, failure to thrive, she died during the procedure and permission for nec- orthopncea, and dyspncea. There was no history ropsy could not be obtained. suggestive of rheumatic fever or disseminated lupus erythematosus. Case 2. A boy of 7 months was admitted in 1952 with a history of cough and failure to thrive. He was CASE REPORTS pale and wasted, weighing 10 lb. 8 oz. (4762 g.). The heart was Case 1. As an infant this girl had repeated pulmonary enlarged. Presystolic and systolic murmurs infections and was noted to have an abnormal heart. were heard at the apex of the heart. Dyspnoea and signs of congestive heart failure were When investigated in 1951 at the age of 4 years she was only temporarily 30 lb. She had differential relieved by digitalis and mersalyl. Radiographs showed http://heart.bmj.com/ small (weight (13-6 kg.)). cardiac enlargement with vascular engorgement cyanosis with clubbing of her toes but pink, normal of the The heart was in sinus lung fields, and the electrocardiogram (Fig. 1) showed finger-tips. rhythm, slightly changes suggestive of right enlarged, and there was a diastolic thrill at the apex. In ventricular hypertrophy. area was an loud first Venous angiocardiography supported the diagnosis of the mitral there extremely sound, mitral stenosis, as the and mid-diastolic and presystolic murmurs, strongly left atrium was large and slow to The empty. The mitral valve was dilated surgically, but suggestive of mitral stenosis. electrocardiogram 36 (Fig. 1) and radiological appearances were consistent hours later the child choked and died almost immedi- with the diagnosis. Venous angiocardiography showed ately. At necropsy (Dr. H. S. Baar) the diagnosis of into the mitral stenosis was confirmed. There was endocardial on October 3, 2021 by guest. Protected copyright. that the right ventricle emptied normally pul- fibro-elastosis monary artery, from which contrast medium passed of the left atrium and upper part of the directly to the descending aorta, confirming reversed left ventricle, with widespread atelectatic areas in both flow through a ductus arteriosus. The left atrium was lungs. to In greatly enlarged and extremely slow empty. 1952 (Cases 1 and 2 were included in the report by Bower the mitral valve was dilated by the digital method. The et al., 1953.) cusps tore rather than split; the valve felt leathery and appeared to have no commissures. The pulmonary Case 3. This boy was noticed to have a murmur at arterial pressure, taken with a saline manometer at the the age of 9 months; he had had recurrent bronchitis and time of operation, was 145 cm. As valvotomy had little had failed to thrive. When investigated in 1951 at the effect on this pressure it was decided not to close the age of 3 years he was small (weight 25 lb. (11-3 kg.), ductus arteriosus. Biopsy of the left atrial appendix 'height 32 in. (81 cm.)), with a proinent sternum and showed moderate fibro-elastosis of the endocardium. recession of the lower ribs. He was dyspnoeic, but not Considerable symptomatic improvement followed and cyanosed. The heart was in sinus rhythm with a tap- was maintained for several years. Then breathlessness ping apical impulse. The mitral first and pulmonary gradually increased and differential cyanosis became second sounds were loud, and a short early systolic mur- Received March 11, 1966. mur, a presystolic murmur, and a mid-diastolic murmur * In receipt of a research grant from the Endowment Fund were heard at the apex. The electrocardiogram showed of the United Birmingham Hospitals. bifid P waves in V3 and evidence of right ventricular 83 Br Heart J: first published as 10.1136/hrt.29.1.83 on 1 January 1967. Downloaded from 84 ~~~~Singh, Gotsman, Abrams, Astley, Parsons, and Roberts CASE NUMBER 2 3 4 5 67 8 ........~~.Aj .r At9 Y .tt:...77.....ttt~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~...... II~~~~~~~~~~~~~7 -~~~~~~~~~~~~~~~~.............. V1 AN#"L cxYL -A-Ai'~" 4~~~~~~~~~~~~~~~~~~~~~~~ F~~~..'- ______ I~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..I V3 A....... .7 7 7 \~~~~~~~~~~~~~~~~~~~~j~~~~~~~~~~~~~~~~~~~I~~~~~~~~~~~~~..... http://heart.bmj.com/ .._ I ... ... I~~~~~~~~~~~~~~ .....Vi.... ________________~~~~~~~~~~~~~ ~~~~~~~~~~~...... on October 3, 2021 by guest. Protected copyright. v~~~FG .TeeetoadormHE n8csso ogntlmta tnss hypertrophy(Fig.1).Enlargement of the right yen- cotomy was undertaken but theand~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.............left atrium was small tricleandleft atrium was confirmed byThe.pulmonarradiograph, the the intended valvotomy was abandoned. pulmonaryartery segment was full and the lung vessels arterial pressure was 40/15 mm. Hg. Heart failure werecongstedAtheae of5 anexporatry tora- deveopedbut as cntrlledwithdigialisand iureics Br Heart J: first published as 10.1136/hrt.29.1.83 on 1 January 1967. Downloaded from Congenital Mitral Stenosis 85 At the age of 10 catheterization was repeated. The pul- revealed an enlarged heart with a prominent left atrium monary wedge pressure was 18 mm. Hg and pulmonary (Fig. 2). The lung vessels were congested and lym- arterial pressure 50/15 mm. Hg. Selective right ven- phatic lines were present. The pulmonary arterial tricular cine-angiocardiography showed a large, slowly pressure was 45-50/22 mm. Hg and the wedge pressure emptying left atrium and a left ventricle of normal size. 20 mm. Hg. In 1962 at the age of 16 years mitral valvotomy was In 1964 a mitral valvotomy was performed under performed under direct vision using cardiopulmonary direct vision, using cardio-pulmonary bypass. Biopsy bypass. This reduced the left atrial pressure from 40/2 of the left atrium showed slight hypertrophy of the myo- to 15/2 mm. Hg. Three years after the operation he cardial fibres and a little endocardial sclerosis. The had no symptoms and the only abnormal sign was a child's immediate post-operative state was satisfactory, faint apical systolic murmur. but he developed mitral regurgitation which became Biopsy of the atrial appendix showed moderate myo- severe. This was successfully corrected at a second cardial hypertrophy but no endocardial fibro-elastosis. operation 12 weeks later. Evidence of pulmonary hypertension and associated breathlessness gradually diminished in the weeks following operation and pro- Case 4. This boy had several attacks of broncho- gressed to complete freedom from symptoms, full exer- pneumonia in infancy and a murmur was heard at the cise tolerance, and a heart of normal size with normal age of 4 months. In 1963, when he was 4 years, he sounds and only a faint short systolic murmur at the was investigated because of breathlessness on exertion, apex. and orthopncea. He was small (weight 34 lb. (15.4 kg.), height 38 in. (96-5 cm.)). The heart was regular, rapid, with a tapping apical impulse, a loud apical first sound, Case 5. This girl was first seen at the age of 3 months and accentuation of the second sound. There was a because she had failed to thrive. She had a persistent rumbling mitral mid-diastolic, a presystolic, and a faint ductus arteriosus which was closed when she was 4 short, early systolic murmur. The electrocardiogram months old. She was readmitted in 1963 at the age of showed evidence of right ventricular hypertrophy and nearly 5 years with congestive heart failure and suspected bifid P waves in lead V3 (Fig. 1). The chest radiograph mitral stenosis. Response to digitalis and diuretics was satisfactory. She was small (weight 30 lb. (13.6 kg.), height 38 in. (96-5 cm.)), with a malar flush and a chest deformity consisting of a prominent sternum and in- drawn lower ribs. The cardiac impulse was of right ventricular type. Tachycardia made it difficult to time the murmurs, but phonocardiography showed that, in addition to loud first mitral and second pulmonary sounds, there was a presystolic murmur at the apex http://heart.bmj.com/ and an early diastolic murmur at the left sternal edge. Electrocardiogram showed sinus rhythm, bifid P waves in V3, and evidence of right ventricular hypertrophy (Fig. 1). Radiography showed cardiomegaly, a promin- ent left atrium, dilated pulmonary artery, and pulmonary vascular congestion with lymphatic lines (Fig. 3). Mean left atrial pressure, measured in 1964 by transseptal punc- ture from the right atrium, was 15 mm. Hg, and the pulmonary arterial pressure was 92/50 mm. Hg. Cine- on October 3, 2021 by guest. Protected copyright. angiocardiography showed a large, slowly emptying left atrium. The mitral valve bulged into the left ventricle during left ventricular filling and was flattened or con- cave during ventricular systole, confiming the diagnosis of mitral stenosis. At operation in 1965 the valve was funnel-shaped with thick rubbery cusps. Its orifice measured 10 cm. in diameter, and in the area where the postero-medial com- missure should have been the cusps were fused to anomalous chordse tendinem. After splitting the valve under direct vision, the orifice measured 2X5 cm. in diameter but left atrial pressure remained high (14 mm.