Acquired supravalvar membranous stenosis of the left atrioventricular valve.

Acquired supravalvar membranous stenosis of the left atrioventricular valve developed in a nine year old boy with atrioventricular discordance. The supravalvar membrane had not been present when a partial annuloplasty was performed on the left atrioventricular valve eight years before. The stenosis was relieved by operative excision of the membrane.     

Sudden death in a youth. A case of quadricuspid aortic valve with isolation of origin of left coronary artery.

A youth of 16 years of age died suddenly and quite unexpectedly while walking to school. The necropsy disclosed a quadricuspid aortic valve with complete isolation of the left coronary artery by an adherent aortic valve cusp. The left ventricular myocardium showed subendocardial contraction band necrosis suggesting that critical ischaemia had triggered a state of hypercontraction, in keeping with ventricular fibrillation as the immediate cause of death. The site of the anomaly in the aortic root showed dysplasia of the aortic wall and the affected valve cusp, histologically similar to the changes that characterise supravalvar aortic stenosis. The findings suggest a developmental anomaly, possibly a forme fruste of supravalvar aortic stenosis, rather than a postnatally acquired condition.     

Williams syndrome associated with complete atrioventricular septal defect

Williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the syndrome. Complete atrioventricular septal defect has rarely been associated with Williams syndrome and only one necropsy case has been reported in the literature. The long term follow up of Williams syndrome associated with complete atrioventricular septal defect is reported. During a 10 year follow up period, the pressure gradient in the ascending aorta did not increase despite narrowing of the ascending aorta as identified on an aortogram.     

Supravalvar aortic stenosis: clinical and hemodynamic profile,and surgical outcome

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.     

Successful cryoablation in the noncoronary aortic cusp for a left anteroseptal accessory pathway

Catheter ablation of anteroseptal accessory pathways may be difficult because of the neighboring conduction tissue that may be damaged with ablation. We report a case of an accessory pathway localized to the anteroseptal region. A pathway potential found in the noncoronary cusp of the aortic valve was successfully targeted for ablation with cryo energy. Observations during tachycardia and pacing maneuvers suggest that the supravalvar aortic musculature may be an integral component of left anteroseptal pathways that can be safely targeted for cryoablation without injury of the atrioventricular conduction system.     

A hammock in the left atrium

Shone's anomaly describes a complex involving multiple left sided cardiac obstructions, namely, parachute deformity of the mitral valve, supravalvular ring of the left atrium, subaortic stenosis and aortic coarctation. We are reporting a case of Shone's anomaly characterised by aortic recoarctation, mitral supravalvular membrane, bicuspid aortic valve, complicated complete atrioventricular block and bradycardia-induced nonsustained polymorphic ventricular tachycardia. We revealed mitral supravalvular membrane by 3D transoesophageal echocardiography.     

Supravalvar mitral stenosis: risk factors for recurrence or death after resection.

OBJECTIVE--To assess the medium term outcome in infants and children after surgical resection of supravalvar mitral stenosis with special reference to risk factors for mortality or recurrence of supravalvar mitral stenosis. No detailed follow up has been previously reported in this uncommon condition. DESIGN--Prospective cross sectional clinical and echocardiographic follow up. SETTING--Paediatric cardiothoracic unit. PATIENTS AND METHODS--23 consecutive children (14 male, nine female, mean age 3 years 2 months at surgery) who underwent resection of supravalvar mitral stenosis between 1978 and 1993. RESULTS--Follow up was for a mean of 58 months (range 0.5-167) after resection of supravalvar mitral stenosis. Four patients developed recurrent supravalvar mitral stenosis: this has not been reported previously. This was recognised 14-108 months after resection and confirmed at repeat operation. Three of these patients had successful reoperations but one died. Five other patients died. On multivariate analysis the only variable associated with survival free of recurrent supravalvar mitral stenosis was older age (18 months or more) at time of surgery (hazard ratio 0.17, 95% confidence interval (CI) 0.03 to 0.95, P < 0.05). Five year actuarial survival free of recurrent obstruction when supravalvar mitral stenosis was resected at age less than 18 months was only 39% (95% CI 9 to 69%) compared with 73% (95% CI 24 to 93%) in older patients. CONCLUSION--Supravalvar mitral stenosis is part of a spectrum of obstructive lesions affecting the left heart. Recurrent supravalvar mitral stenosis can develop after surgical resection. The prognosis in those who require resection within the first 18 months of life is poor: mortality is high, as is the risk of recurrent supravalvar mitral stenosis in survivors, probably because of continuing turbulent flow across a small left ventricular inflow tract.     

Quantitative evaluation of complete endocardial cushion defect using two-dimensional echocardiography

To avoid postoperative mitral valve dysfunction, the common atrioventricular valves of 21 preoperative patients with complete common atrioventricular canal were quantitatively evaluated using two-dimensional echocardiography. The criteria for diagnosing hypoplasia of the left lateral leaflet, which often made complete repair difficult, were also investigated. The patients were 10 boys and 11 girls, who ranged in age from one month to three years and three months. The subcostal short-axis view was used to evaluate the common atrioventricular valves. The sizes of the leaflets and diameters of the ventricles were measured by two-dimensional echocardiography and at surgery. The measurements by both methods were nearly identical, and the correlation coefficient was 0.95. Thus, two-dimensional echocardiography correctly evaluated common atrioventricular valve size. The length of the left lateral leaflet varied from 5 mm to 22 mm. In four patients, it was less than 10 mm, and was under the 99% confidence limit. In all four patients, two papillary muscles were noted in the left ventricle. Two of these patients underwent corrective surgery and died of postoperative mitral valve stenosis. This new technique can prevent postoperative mitral valve dysfunction by estimating preoperatively the appropriate suturing length of the anterior and posterior components of the anterior mitral valve. It is concluded that two-dimensional echocardiography can correctly evaluate the size of common atrioventricular valves, which is often difficult by angiocardiography. Hypoplasia of the left lateral leaflet could be diagnosed when its length was less than 10 mm measured by two-dimensional echocardiography. This technique is more accurate compared to the method which demonstrates the presence of a single papillary muscle in the left ventricle. Corrective surgery for the patients with a hypoplastic left lateral leaflet involves great risks, resulting in postoperative mitral valve stenosis. Preoperative evaluation of a common atrioventricular valve is useful to avoid postoperative mitral valve dysfunction.     

Double inlet left ventricle

Twenty-five cases of double-inlet left ventricle, 23 in atrial situs solitus and 2 with dextroisomerism are described. Twenty had both atrioventricular valves and in 3 a common atrioventricular valve connected to the left ventricle. In rest, the left atrioventricular valve was straddling over the left-sided right ventricle in about 15%. The position of the rudimentary right ventricle varied from superior, anterior and to the right of left ventricle (6 with transposition and 9 with concordant ventriculo arterial connection), to superior anterior and to the left of left ventricle (10 cases, all with transposition). Five cases had stenosis of the left atrioventricular valve and 2 stenosis of the right one. In 11, the clinical presentation was dominated by cyanosis, reduced pulmonary blood flow and right to left shunt. The rest had cyanosis and congestive heart failure. Five patients with right ventricle on the left had complete atrioventricular block, 9 right AQRS orientation and all of them findings of left ventricle hypertrophy. Three cases showed initial Q wave in VI and one left bundle branch block. Cases with right ventricle on the right, had left QRS orientation, left ventricle hypertrophy and 2 complete atrioventricular block. Cross-sectional echoes showed in 13, two atrioventricular valves committed to the left ventricle; in 3 of them the right ventricle was visualized. In 22 cases angiography determined the type of atrioventricular connection. Double inlet left ventricle is one of the most challenging diagnosis in congenital heart disease. Considering its lack of clinical specificity and according to our results, the electrocardiogram, echocardiogram and angiographic findings are of great help for its identification.     

Univentricular atrioventricular connection to a dominant left ventricle with a concordant ventriculo-arterial connection

Double inlet left ventricle with concordant ventriculo-arterial connection, first described by Holmes, is an unusual cardiac malformation which includes a right-sided rudimentary right ventricle and, frequently, subpulmonary stenosis. We have now encountered six patients, aged 1 month to 13 years, with this basic combination. In 5 cases, cross-sectional echocardiography, using parasternal, apical and subcostal views was diagnostic. Subpulmonary stenosis was seen in two of them. The sixth case shown to have atresia of the left atrioventricular valve, a concordant ventriculo-arterial connection, a left-sided rudimentary right ventricle, mild subpulmonary stenosis and a right-sided aorta. The combination of the left atrioventricular valve, left-sided rudimentary right ventricle and a concordant ventriculo-arterial connection has not, to our knowledge, been previously reported. Cross-sectional echocardiography always provided the correct morphologic diagnosis.     

Routine cleft closure in repair of complete atrioventricular septal defects

Thirty patients with complete atrioventricular septal defect were operated upon between 1989 and 1996 at our institute. Their ages ranged from two months to 24 years (mean age 2.81 years). All patients had severe pulmonary arterial hypertension except one who had associated pulmonic stenosis. Four patients had severe atrioventricular valve regurgitation and another six had moderate regurgitation. Five patients had Down's syndrome. The cleft in the left atrioventricular valve was closed in all but seven patients. There were five (16.66%) in-hospital deaths which included two patients in whom the cleft was left open. Follow-up ranged from three months to eight years. All patients were evaluated by 2D echocardiography in the immediate post-operative period and three to six monthly subsequently. One patient progressed to moderate and another to severe left atrioventricular valve regurgitation during follow-up. In both the patients the cleft had been left open. All other patients continue to have stable left atrioventricular valve status, that is, none or trivial to mild regurgitation. In view of late deterioration of the left atrioventricular valve regurgitation only in those patients where the cleft was left open, we suggest complete closure of the cleft in all complete atrioventricular canal defects.     

Cardiovascular malformations in Turner's and Noonan's syndrome

The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies.     

Cardiovascular malformations in Turner's and Noonan's syndrome.

The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies.     

Isolated Supravalvar Aortic Stenosis Without William's Syndrome

Supravalvular aortic stenosis, characterized by narrowing of the ascending aorta above the valve, is the least common form of left ventricular outflow tract obstruction and is usually associated with William''s syndrome. We present a case of a 27-year-old male with isolated supravalvar aortic stenosis (SVAS) presenting with heart failure. This case underscores the fact that in rare cases sporadic SVAS can occur in isolation without the classic findings of William''s syndrome and highlighting the importance of integration of clinical and echocardiographic recognition for definitive management.     

Signs of membranous subaortic stenosis appearing after correction of persistent common atrioventricular canal

Two cases are presented of persistent common atrioventricular (A-V) canal with interventricular communication in which associated membranous subaortic stenosis became apparent only after correction of the basic malformation and replacement of the mitral valve. The features of the membranous subaortic stenosis present only after operation were a left ventricular outflow pressure gradient and angiocardiographic demonstration of the membrane. In one case, additional echocardiograms demonstrated systolic flutter of the aortic cusps after the first operation (no echocardiograms were taken preoperatively). The flutter disappeared after a second operation in which the subaortic membranous tissue was resected. The appearance of hemodynamic signs of subaortic stenosis after the corrective operation for the persistent common A-V canal is explained by the fact that preoperatively the interventricular communication allowed decompression of the left ventricle so as to preclude a left ventricular outflow gradient. It Is presumed that the use of additional angiocardiographic projections might have demonstrated the anatomic state preoperatively.     

完全性房室隔缺损的手术治疗(附36例报告)

目的探讨完全性房室隔缺损的治疗方法。方法在中度低温体外循环下采用双补片法修复36例完全性房室隔缺损。结果手术死亡2例，余患者术后均恢复为窦性心律，无完全性房室传导阻滞。复查心脏彩超二尖瓣轻度反流8例，中度反流2例。结论完全性房室隔缺损应尽早手术。双补片法效果较好。术中应注意预防左室流出道狭窄和矫治二尖瓣关闭不全。     

Double inlet left ventricle. Morphopathology and surgical anatomy

Because of the possibility of surgical treatment of double inlet left ventricle, its basic morphologic features of surgical and imaging importance are analyzed. Seventeen hearts were studied with the segmental sequential system. The situs was solitus in thirteen; dextroisomerism in three and levoisomerism in one. The atrioventricular valves were separated in nine; there was a common atrioventricular valve in eight; straddling of the atrioventricular valve in seven and stenosis in two. The rudimentary right ventricle was to the right side in twelve hearts and to the left side in five. The discordant ventriculoarterial connection was the most frequent (seven), followed by the concordant one (five) and double outlet right ventricle (five). There was pulmonary stenosis in five. The ventricular septum did not reach the crux cordis. The ventricular septal defect was bigger in straddling atrioventricular valves than in valves completely open into the left ventricle, being restrictive in ventriculoarterial concordance. Surgical treatment varies as the complexity of this cardiopathy does, from cardiac septation to palliation procedures. The morphologic knowledge of this cardiac malformation is basic to interpret correctly the diagnostic imaging.     

Double outlet right ventricle with atrioventricular discordance. Report on cases having situs solitus, dextrocardia, pulmonary stenosis and levomalposition of the aorta

We describe six patients with situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, ventricular septal defect, double outlet from the morphological right ventricle, pulmonic stenosis and levomalposition of the aorta. Four patients were male and two female; their age ranged from 3.5 to 31 years (mean 13.8 years). All had various degrees of disability, and presented with cyanosis, clubbing and high hematocrit levels. One patient had an atrio-ventricular block that varied from first to third degree; another patient showed intermittent junctional rhythm. At angiography the ventricular septum appeared to be almost perpendicular to the frontal plane in most cases, so that the anteroposterior projection resulted in a true axial view. One overriding left atrioventricular valve and one straddling right atrio-ventricular valve were demonstrated; no significant incompetence of either valve was observed. The ventricular septal defect was always single and related to the subpulmonary outflow. Pulmonic stenosis was valvular in every patient; an additional infundibular obstruction was present in one case. In two cases an additional stenosis was discovered at the supravalvular level. The left pulmonary branch was stenotic in one case; it was hypoplastic, with controlateral dilatation, in two cases; both pulmonary arteries were dilated in one case. The aorta was always to the left of the pulmonary artery, either anterior or side by side. Three patients were operated on in different Institutions: one had a pulmonic valvotomy at the age of six years; one, aged twenty, had a right Blalock-Taussig shunt; the third, with overriding left atrioventricular valve, underwent a modified Fontan operation at the age of thirty years with success.(ABSTRACT TRUNCATED AT 250 WORDS)     

Echocardiographic evaluation of congenital mitral valve anomalies in children

Congenital mitral valve anomalies were diagnosed in 65 children, whose ages ranged from newborn to 18 years, using 2-dimensional, color, pulsed-, and continuous-wave Doppler ultrasound. Data were collected over 7.5 years from 13,400 new studies. Data in these patients were compared with those obtained by cardiac catheterization, cardiac surgery, and autopsy. We detected 4 different lesions: (1) congenital mitral stenosis with 2 papillary muscles (n = 24); (2) parachute mitral valve, with a single papillary muscle (n = 24); (3) isolated cleft in the mitral valve (n = 10); and (4) double-orifice mitral valve (n = 7). A supravalvar mitral ring was detected in 21 patients with mitral stenosis; however, it never occurred as an isolated lesion and was invariably associated with some other left ventricular inflow or outflow obstruction. The supravalvar ring was associated with a parachute deformity of the mitral valve in 17 patients; in only 4 was this abnormality associated with mitral stenosis with 2 papillary muscles. In patients with congenital mitral stenosis, the peak and mean transmitral Doppler velocities were increased significantly compared with those in controls (peak velocity 1.53 ± 0.74 vs 0.86 ± 0.25 m/s, respectively, p < 0.01; mean velocity 1.13 ± 0.61 vs 0.58 ± 0.11 m/s, respectively, p < 0.01). The correlation between mean transmitral pressure gradient obtained by Doppler and cardiac catheterization was fair (r = 0.75). However, the correlation between the mitral valve areas calculated by the Doppler pressure half-time method and by the Gorlin formula was poor (r = 0.57). Eleven of the 24 patients with parachute mitral valve deformities had Doppler evidence of an increased transmitral gradient. In children, echocardiography proved superior to cineangiography as a diagnostic modality, and can be relied upon in making management decisions, but Doppler velocities, including pressure halftime, applicable in adults for calculation of mitral valve area are unreliable.     

Quantitative angiocardiographic recognition of atypical form of tetralogy of Fallot with absent pulmonic valve

A 9-year-old boy is reported who had tetralogy of Fallot, rudimentary pulmonic valve, and supravalvar pulmonic stenosis. The patient lacked almost all of the clinical findings commonly encountered in this syndrome. Thus he never had signs of a large left-to-right shunt, or congestive heart failure. He had no murmur of pulmonic regurgitation and his pulmonary artery was not large on chest roentgenogram. Angiocardiography revealed moderate main pulmonary arterial dilation. The lack of the usual manifestations of tetralogy of Fallot with absent pulmonic valve was due to supravalvar pulmonic stenosis, acting as a natural pulmonary artery band. A quantitative angiocardiographic study was undertaken in order to find a clue for the diagnosis of these atypical cases. The ratio of the transverse diameters of the main pulmonary artery and the aortic root as measured on lateral angiocardiograms in 31 children with uncomplicated tetralogy of Fallot was 0.70 +/- 0.22, whereas this ratio was 1.70 in this particular patient (p less than 0.001). It is concluded that a quantitative evaluation of the ratio of the transverse diameter of the main pulmonary artery to the aortic root on lateral angiocardiogram allows differentiation of tetralogy of Fallot with absent pulmonic valve associated with supravalvular pulmonic stenosis from uncomplicated forms of tetralogy of Fallot.