Ventricular septal defect with congenital mitral valve disease: long-term results of corrective surgery

BACKGROUND: A retrospective analysis of the mortality, morbidity and long-term follow-up of patients undergoing corrective surgery for ventricular septal defect and congenital mitral valve disease is presented. METHODS AND RESULTS: Between January 1991 and December 2000, 69 consecutive patients aged 2 months to 45 years (median 18 months) underwent repair of ventricular septal defect and associated mitral valve disease. In 52 patients (75%), the ventricular septal defects were located in the perimembranous and subarterial area. Forty-six patients had congenital mitral incompetence and 23 had congenital mitral stenosis. The ventricular septal defect was repaired through the right atrium in all. Sixty-five patients underwent reconstruction of the mitral valve and 4 underwent primary mitral valve replacement. Another 4 patients underwent mitral valve replacement after a failed repair. Associated procedures included: patent ductus arteriosus ligation (n=12), aortic valve replacement (n=6), coarctation repair (n=13), interrupted aortic arch repair (n=1), atrial septal defect closure (n=17) and Takeuchi repair (n=1). There were 6 early deaths (8.6%). Three deaths were due to pulmonary arterial hypertensive crisis and one due to residual mitral stenosis. One death was due to intractable congestive heart failure. Another patient died due to persistent low cardiac output. Follow-up ranged from 6 months to 120 months (mean 64.4+/-33.6 months). Reoperation was required in 22 patients, mainly for recurrent/residual mitral valve dysfunction or hemodynamically significant left ventricular outflow tract obstruction. There were 4 late deaths, 2 due to residual mitral stenosis and the other 2 as a result of a thrombosed prosthetic valve. At 10 years, the actuarial survival rate was 850+/-5.0%, and freedom from reoperation was 45%+/-10.0%. CONCLUSIONS: Reconstruction of the mitral valve along with closure of VSD is possible in most cases. However, careful follow-up is recommended to detect changes in the mitral valve status over a course of time.     

The right heart in congenital heart disease

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.     

Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography

Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of greater than or equal to 16 mm Hg at any level of the pulmonary venous return or of the systemic ventricular inflow. Severe obstruction was predicted if Doppler examination measured a flow velocity of greater than or equal to 2 m/s across any area of inflow obstruction. At catheterization, 12 patients (39%) had severe obstruction to left ventricular inflow or pulmonary venous return and all obstructions were correctly detected by Doppler echocardiography. The site of pulmonary venous obstruction was localized by two-dimensionally directed pulsed Doppler study. Patients with a lesser degree of obstruction had a lower Doppler velocity, but none had a maximal Doppler velocity of greater than or equal to 2 ms/s.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cor Triatriatum Sinistrum Presenting as Cardioembolic Stroke: An Unusual Cause of Adolescent Hemiparesis

Cor triatriatum sinistrum is a rare congenital cardiac malformation, in which the left atrium (LA) is divided into two distinct chambers by a fibromuscular membrane. Classically, the proximal (upper or superior) chamber of the LA receives pulmonary venous connections, whereas the distal (lower or inferior) chamber contains LA appendage and true atrial septum containing fossa ovalis. The distal chamber is in continuity with the atrioventricular valve, while the two chambers communicate through a defect in the membrane. The hemodynamics of cor triatriatum sinistrum are similar to that of mitral stenosis due to obstructive property of membrane. The majority of reported cases of cor triatriatum occur in infants with symptoms of pulmonary venous obstruction, with adult cases being rare. Herein, we describe an unusual case of cor triatriatum in a 17‐year‐old boy who presented for the first time with embolic cerebral infarction with left hemiparesis.     

Cor triatriatum with mitral valve disease in adults

One 16-year-old boy with cor triatriatum and congenital mitral regurgitation and two women, 35- and 54-years-old, with cor triatriatum and rheumatic mitral stenosis are reported. The regurgitant mitral valve in the boy had three papillary muscles and short chordae tendineae. One of the patients with rheumatic mitral stenosis had a subtotal cor triatriatum with enlargement of the left atrial appendage and without asymmetry in the signs of pulmonary congestion. The mitral regurgitation facilitated the angiographic diagnosis of cor triatriatum. However, neither of the two patients associated with rheumatic mitral stenosis were correctly diagnosed preoperatively. All three patients were operated on with satisfactory results.     

Results of the arterial switch operation in patients with transposition of the great arteries and abnormalities of the mitral valve or left ventricular outflow tract

Between January 1983 and October 1989, 290 patients underwent an arterial switch operation for transposition of the great arteries; 30 (10.3%) of the patients had abnormalities of the left ventricular outflow tract or mitral valve, or both. These abnormalities included isolated pulmonary valve stenosis (n = 9), septal (dynamic) subpulmonary stenosis (n = 5), anatomic (fixed) subpulmonary stenosis (n = 7), abnormal mitral chordae attachments (n = 2) or a combination of abnormalities (n = 7). There were two early deaths, one of which was due to previously unrecognized mitral stenosis and a subpulmonary (neo-aortic) membrane and one late death due to presumed coronary obstruction. Of the nine patients with pulmonary valve abnormalities due to either a bicommissural (n = 5) or a thickened tricommissural (n = 4) valve, only one underwent valvotomy. Peak systolic ejection gradients in these nine patients measured preoperatively ranged from 0 to 50 mm Hg. At follow-up study 5 to 30 months postoperatively, the neo-aortic valve gradient was less than or equal to 15 mm Hg in all patients; three patients had mild neo-aortic regurgitation. Preoperative gradients may overestimate the degree of obstruction because of the increased pulmonary blood flow present in transposition. No patient with "dynamic" subpulmonary obstruction before the arterial switch operation had a surgical procedure performed on the left ventricular outflow tract; none had evidence of subaortic obstruction after the arterial switch.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

23例三房心临床分析

目的：总结三房心临床特点、合并畸形及影像学诊断特点。方法：1993年1月至2005年6月共收治23例三房心患者,男13例,女10例,年龄0．3～38（9．6±10．0）岁。均为左型三房心,其中21例获得手术证实,完全型15例,部分型6例。心电图检查：电轴右偏18例,电轴偏左1例,电轴正常4例。胸片检查：18例肺血增多。结果：术前经超声检查确诊12例（52．2％）,10例行心导管确诊4例（40％）。21例中16例（76．2％）合并其它畸形,合并房间隔缺损14例（66．7％）、部分肺静脉异位引流6例（28．6％）、肺动脉瓣狭窄3例（14．3％）,永存左上腔3例（14．3％）。结论：三房心临床表现多样,合并畸形发生率较高,彩色超声心动图检查有较高诊断价值。心导管检查对合并畸形诊断率较高,对合并畸形者强调术中探查。     

Cor triatriatum sinistrum. Diagnostic features on cross sectional echocardiography.

M mode and cross sectional echocardiography was carried out in three cases of cor triatriatum sinistrum (two infants and one adult). In two cases a peculiar double arch appearance, not previously reported, was found. All three cases were referred for surgery without cardiac catheterisation, and the diagnosis proved to be correct. The characteristic echocardiographic feature of cor triatriatum is an intra-atrial membrane detected in multiple planes of examination, curving anteroinferiorly and inserting some distance away from the mitral valve ring, proximal to the left atrial appendage. Superiorly the membrane runs parallel to, and a short distance behind, the aortic root creating a superior recess of the distal left atrial chamber. These features differentiate cor triatriatum from a supravalvar mitral ring. During diastole the membrane moves forward towards the mitral valve funnel. This, together with the arching appearance of the membrane on four chamber views and the more superior position of the membrane, makes it possible to distinguish cor triatriatum from total anomalous pulmonary venous drainage to the coronary sinus. From a review of past experience at the Brompton Hospital of the diagnostic accuracy of cardiac catheterisation in this condition, it is concluded that cross sectional echocardiography is superior to angiography as a technique for diagnosing cor triatriatum.     

Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham''s chorea: diagnosis and preoperative assessment by cross sectional echocardiography

In cor triatriatum sinister, one of the rarest congenital cardiac anomalies, a membrane divides the left atrium into a pulmonary venous component above and the vestibule below. The importance of the anomaly lies in the effects of the resultant pulmonary venous obstruction that usually present in the first year of life and can mimic obstructed total anomalous venous drainage or congenital mitral stenosis. A case presented as mitral stenosis in the third decade of life, ten years after a well documented episode of Sydenham's chorea. The diagnosis was made rapidly by transthoracic echocardiography and transoesophageal echocardiography was used for complete assessment. Cardiac catheterisation added nothing to the non-invasive diagnosis or the preoperative assessment. Uncomplicated corrective surgery was undertaken.     

Cor triatriatum sinistrum. Diagnostic features on cross sectional echocardiography

M mode and cross sectional echocardiography was carried out in three cases of cor triatriatum sinistrum (two infants and one adult). In two cases a peculiar double arch appearance, not previously reported, was found. All three cases were referred for surgery without cardiac catheterisation, and the diagnosis proved to be correct. The characteristic echocardiographic feature of cor triatriatum is an intra-atrial membrane detected in multiple planes of examination, curving anteroinferiorly and inserting some distance away from the mitral valve ring, proximal to the left atrial appendage. Superiorly the membrane runs parallel to, and a short distance behind, the aortic root creating a superior recess of the distal left atrial chamber. These features differentiate cor triatriatum from a supravalvar mitral ring. During diastole the membrane moves forward towards the mitral valve funnel. This, together with the arching appearance of the membrane on four chamber views and the more superior position of the membrane, makes it possible to distinguish cor triatriatum from total anomalous pulmonary venous drainage to the coronary sinus. From a review of past experience at the Brompton Hospital of the diagnostic accuracy of cardiac catheterisation in this condition, it is concluded that cross sectional echocardiography is superior to angiography as a technique for diagnosing cor triatriatum.     

Surgical case of partial anomalous pulmonary venous connection to superior vena cava with cor triatriatum: Williams’ modification and excision of diaphragm

We experienced a successful pediatric surgical case of partial anomalous pulmonary venous connection to the superior vena cava with cor triatriatum. Echocardiography and multidetector-row computed tomography showed partial anomalous pulmonary venous connection (right upper pulmonary vein connected to the high superior vena cava) and atypical cor triatriatum (analogue to type III-A2 of Lucas-Schmidt classification: left upper pulmonary vein had dual connection to the innominate vein via vertical vein and the accessory chamber). At 8 years of age, the male patient underwent extracardiac right atrial pedicle repair of partial anomalous pulmonary venous connection to the superior vena cava (Williams’ modification) and excision of the diaphragm between the accessory chamber and the left atrium simultaneously. The postoperative course was uneventful in normal sinus rhythm and there was no stenosis of newer drainage root from right upper pulmonary vein.     

Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment by cross sectional echocardiography.

In cor triatriatum sinister, one of the rarest congenital cardiac anomalies, a membrane divides the left atrium into a pulmonary venous component above and the vestibule below. The importance of the anomaly lies in the effects of the resultant pulmonary venous obstruction that usually present in the first year of life and can mimic obstructed total anomalous venous drainage or congenital mitral stenosis. A case presented as mitral stenosis in the third decade of life, ten years after a well documented episode of Sydenham's chorea. The diagnosis was made rapidly by transthoracic echocardiography and transoesophageal echocardiography was used for complete assessment. Cardiac catheterisation added nothing to the non-invasive diagnosis or the preoperative assessment. Uncomplicated corrective surgery was undertaken.     

Unusual case of new‐onset heart failure due to cor triatriatum sinister

We report the case of a 30‐year old man who came to the emergency department of our hospital with acute left heart failure, and was diagnosed with a rare congenital anomaly (cor triatriatrum sinister), which can mimic a severe mitral stenosis. Cor triatriatum sinister is a rare anomaly (0.1% of all cases of congenital heart disease) that is seldom diagnosed in adult patients. The hallmark of this congenital defect is the presence of a fibromuscular membrane that divides the left atrium (LA) into two chambers: a postero‐superior chamber into which the pulmonary veins drain and an infero‐anterior chamber (true LA) containing the mitral valve and atrial appendage. Both chambers communicate through a membrane in which one or more drain holes can be found. When the hole is significantly obstructive, it results in increased venous and arterial pressures. Even though the definitive treatment of cor triatriatum is the surgical excision of the membrane, we present a balloon dilatation case with a good response to percutaneous therapy, both initially and in the ensuing months.     

Membranous supravalvular mitral stenosis: a treatable form of congenital heart disease

The clinical data, echocardiographic findings, operative anatomy and postoperative follow-up were assessed in 14 patients who had surgery for membranous supravalvular mitral stenosis between 1978 and 1985. The patients ranged in age from 6 weeks to 13 years at the time of operation, and 8 of the 14 had associated mitral valve abnormalities. Other associated lesions included ventricular septal defect (n = 7), coarctation of the aorta (n = 5), left superior vena cava (n = 6), subaortic stenosis (n = 3) and atrial septal defect (n = 1). Twelve of the 14 patients had successful removal of the supravalvular membrane, which was usually adherent to the valve, and 2 patients with associated mitral valve abnormalities underwent mitral valve replacement. There were no operative deaths. Review of preoperative two-dimensional echocardiograms, which were available in 11 patients, revealed two types of membranous supravalvular mitral stenosis in 10 patients. In four of these patients, the membrane was only evident after repeated stop action viewing from a single subcostal or parasternal location. The membrane was never seen in one patient. Eleven patients had follow-up in excess of 1 year, and there was one late death. Eight of the remaining 10 patients are asymptomatic, and 7 have no clinical evidence of residual mitral obstruction. Failure to recognize membranous supravalvular mitral stenosis can result in undue delay of cardiac surgery with resultant cardiopulmonary deterioration. Patients with evidence of left ventricular inflow obstruction should have extensive echocardiographic evaluation in an effort to detect membranous supravalvular mitral stenosis, which may be amenable to surgical repair.     

Cardiovascular findings, and clinical course, in patients with Williams syndrome

AIMS: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. METHODS AND RESULTS: The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0.01). Supravalvar aortic stenosis occurred in 32 patients (71%), pulmonary arterial stenosis in 17 (38%), and mitral valvar prolapse in 12 (27%), 9 of these having regurgitant valves. Pulmonary valvar stenosis, ventricular septal defect, coarctation of the aorta, persistent patency of the arterial duct, hypertrophic cardiomyopathy, and subaortic stenosis all occurred less frequently. In 21 patients (47%), 24 surgical or catheter interventions had been made, most often for repair of supravalvar aortic stenosis, undertaken on 16 occasions with just one recurrence, and in 4 along with surgery to the mitral valve. Other lesions requiring intervention were pulmonary valvar stenosis, pulmonary arterial stenosis, coarctation of the aorta, and subaortic stenosis. We lost 3 patients (7%), with severe supravalvar aortic stenosis and moderate or severe mitral regurgitation, 2 early and one late after surgery. CONCLUSION: The most frequent cardiovascular anomalies in Williams syndrome were supravalvar aortic stenosis, pulmonary arterial stenosis, and mitral valvar prolapse, which occurred more frequently in our patients than previously observed. Patients with left ventricular pressure and volume overload were at greater risk.     

Cor triatriatum with persistent levoatrial cardinal vein late presented as severe mitral stenosis

Cor triatriatum is a rare congenital cardiac anomaly that usually becomes symptomatic in the first years of life. We present a 28-year-old pregnant female patient presented with shortness of breath, palpitations and decreased exercise tolerance. Transthoracic (TTE) and transesophageal (TEE) echocardiography showed cor triatriatum, features of severe mitral stenosis and atrial septal defect (ASD). The patient underwent successful surgical correction with an uneventful postoperative course.     

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.     

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.     

Color doppler-echocardiography study of the triatrial heart

Two-dimensional echocardiography has proved its utility in the diagnosis of cor triatriatum sinister by visualizing the defect and its relation to the other structures. Recently pulsed and continuous wave Doppler have been used to determine the degree of obstruction of the membrane. In this study we present three patients with ages six, eight and fourteen months respectively, all with a clinical history of growth retardation, respiratory infections and dyspnea. The patients were studied with two-dimensional and color flow Doppler echocardiography. In all, a membrane was visualized, which divided the left atrium in two chambers, a superior one which received the pulmonary veins and an inferior in continuity with the mitral valve and the left atrial appendage and with color flow Doppler the absence of obstruction at this level was demonstrated. In one patient besides the anomaly of cor triatriatum complete transposition of great arteries with pulmonary stenosis was diagnosed and in another one, patent ductus arteriosus and ventricular septal defect. Diagnosis were confirmed by hemodynamic studies. It is concluded that the color flow Doppler is a diagnostic method of great utility because it permits to obtain more information which complements conventional echocardiography.