Complex Congenital Heart Lesions Research Articles

Employment and insurance for young adults with congenital heart disease.

To determine the life and health insurability and employability of young adults with congenital heart disease. Questionnaire study. Cardiac department of a tertiary referral hospital for children. Young adults 18-30 years old with a variety of congenital heart defects, both simple and complex, including postoperative patients. Availability of insurance at normal or high rates, with or without special conditions or exclusions. Prospects for employment. Questionnaires were sent to eight large life insurance companies, five health insurance companies and, 15 employers, and 26 replies were received (93%). The consensus for life insurability was that young adults with mitral valve prolapse without regurgitation, postoperative ductus arteriosus, and aortic coarctation were insurable at standard rates. Those with any of the other heart defects listed were either insurable at high rates, or in the case of many lesions, not insurable at all. The consensus for health insurance was that insurance was available, but with complete exclusion of benefit for the cardiac disorder. Employment prospects were good for those with simple defects, but poorer for those with complex lesions. Prospects for insurance and employment for young adults with complex congenital heart lesions are poor. Inconsistencies found in insurance and job policies may be due to lack of appropriate guidelines for the outcome of young adults with corrected and uncorrected congenital heart disease.

Pediatric cardiac surgery.

Although there is fair agreement in many areas of pediatric cardiac surgery, issues of controversy and uncertainty persist. The role of the Fontan procedure in the treatment of complex congenital heart lesions is expanding, mainly by necessity, because an alternative biventricular repair is often not available. Reports on medium- and long-term results after Fontan's operation clearly demonstrate the palliative nature of the procedure, even in good candidates, and do not seem to support the widening of its indications. The purpose of this paper is to highlight this controversy, as well as to report on the state of affairs in the treatment of hypoplastic left heart syndrome, transposition of the great arteries, and tetralogy of Fallot.

Extracorporeal membrane oxygenation for cardiac failure after congenital heart operation

Despite continuing improvement in myocardial protection and surgical technique, the repair of complex congenital heart lesions can result in cardiopulmonary compromise refractory to conventional therapy. In a 29-month period, 24 patients (aged 14 hours to 6 years) were treated with extracorporeal membrane oxygenation (ECMO) 28 times for profound cardiopulmonary failure. Four patients required ECMO after each of two cardiopulmonary bypass procedures. Seventeen patients required ECMO to be initiated in the operating room: 12 (71%) were weaned successfully from ECMO, and 8 (47%) survived. Seven patients had ECMO initiated in the intensive care unit: 6 (86%) were weaned, and 5 (71%) survived. Serial echocardiograms demonstrated substantial recovery of cardiac function in 18 of 21 instances (86%) of ventricular failure from myocardial dysfunction. Overall, 18 of 24 patients (75%) were successfully weaned from ECMO including all 4 who underwent 2 ECMO treatments. We conclude that ECMO can successfully salvage children who have serious cardiopulmonary failure immediately after a congenital heart operation and that long-term survival is possible after two ECMO treatments.

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

We reviewed the outcome of 76 consecutive patients (age range, 5 months to 6 years; median age, 19 months) who underwent a modified Fontan procedure after initial palliative surgery for hypoplastic left heart syndrome (HLHS) between January 1984 and December 1989. Modifications of the Fontan procedure included transatrial baffle of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena cava baffle within the right atrium to the superior vena caval-pulmonary artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years). Of the 43 survivors, 25 patients have returned for postoperative cardiac catheterization at a medium of 13 months after the Fontan procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6 l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No patient had significant tricuspid or native pulmonary valve insufficiency. Survival after the Fontan procedure in patients with HLHS is comparable to survival after a Fontan procedure in patients with other complex congenital heart lesions. In the subgroup of patients with HLHS who survived both reconstructive surgery and a Fontan procedure and have been evaluated by cardiac catheterization after a Fontan procedure, the use of the right ventricle as the systemic ventricle yielded excellent intermediate results for Fontan physiology.

Results of total cavopulmonary connection in the treatment of patients with a functional single ventricle

Total cavopulmonary connection was proposed as a modification of the Fontan procedure that might have greater benefits than previous methods. To assess this procedure we reviewed case histories of 38 patients (aged 17 months to 30 years) who underwent Fontan procedures with cavopulmonary anastomoses between January 1987 and December 1989. The group included 32 patients with univentricular heart, 2 with pulmonary atresia and intact ventricular septum, 3 with tricuspid atresia, and 1 with hypoplastic left heart syndrome. One or more previous palliative procedures were performed in 34 patients, including 19 systemic-pulmonary shunts, 16 pulmonary artery bandings, 7 atrial septectomies/septostomies, 7 Glenn shunts, and 1 patent ductus arteriosus ligation. Preoperative hemodynamics showed a pulmonary artery pressure of 12 mm Hg (range 6 to 22 mm Hg), pulmonary-systemic flow ratio of 1.6 (range 0.37 to 3.0), left ventricular end-diastolic pressure 9 mm Hg (range 3 to 15 mm Hg), and systemic arterial oxygen saturation of 82% (range 67% to 94%). Concomitant with cavopulmonary connection, 13 patients underwent additional procedures, including 9 atrioventricular valve annuloplasties, 4 Damus-Stansel-Kaye procedures, and 2 resections of subaortic membranes. Modifying the Fontan procedure in this fashion was particularly useful in the management of 2 patients with pulmonary atresia and intact ventricular septum who had right ventricular-dependent coronary blood flow. Cavopulmonary anastomosis and atrial septectomy were performed in both patients, with resultant inflow of oxygenated blood to the right ventricle and coronary arteries. Excellent postoperative results were noted in each. Postextubation hemodynamics for the entire group included a mean right atrial pressure of 13 mm Hg (range 11 to 17 mm Hg), a mean left atrial pressure of 6 mm Hg (range 3 to 12 mm Hg), and a room air oxygen saturation of 96% (range 92% to 98%). Seven patients had pleural effusions, 3 required postoperative pacemaker placement, and 2 required reoperation for tamponade. A venous assist device was required in one patient on the second postoperative day, but the patient was weaned successfully within 24 hours. One early death (2.6%) occurred in a patient who had intractable ventricular fibrillation 2 days after operation. There was one late cardiac death (2.7%) caused by ventricular failure and one late noncardiac death. These results demonstrate that total cavopulmonary connection provides excellent early definitive treatment, with low morbidity and mortality, for a variety of complex congenital heart lesions.

Double inlet left ventricular main chamber, subaortic small left sided right ventricle and interrupted aortic arch type A. What operation is indicated when?

A case of a 23 year old female patient who suffered from the complex congenital heart lesion of a double inlet left ventricular main chamber, subaortic small left sided right ventricle and interrupted aortic arch type A is reported. With equally high blood pressures, the perfusion in the upper half of the body was maintained through the ascending aorta while the lower half and the lungs were supplied through the pulmonary artery and a patent ductus arteriosus (PDA). Angiographically, the bulbo-ventricular foramen appeared to be nonrestrictive. However, distinct signs of muscular subaortic stenosis were detected. The hemodynamic status principally allowed surgical correction when this became necessary because of increasing left heart failure. Treatment for this complex lesion undoubtedly required reduction of pulmonary perfusion, even when associated with the danger of increasing cyanosis. Various forms of surgical treatment (functional correction, palliative procedures) were discussed. The most elegant was performed without cardiopulmonary bypass: this consisted in connection of the pulmonary artery with the descending aorta using a 16 mm Dacron tube, reconstruction of the aortic arch by a prostheso-subclavian synthetic graft, suture ligation of the PDA, and banding of the pulmonary artery trunk distal to the origin of the prosthesis. One year after the operation, the patient's physical performance has improved. Moreover, despite the disappearance of cardiac failure she has not become more cyanotic during exertion.

Continuous wave Doppler in the evaluation of simple and complex congenital heart disease in infants and children

Continuous wave Doppler assessment of systolic pressure gradients was performed using a digital maximal frequency estimator in 118 consecutive infants and children with suspected ventricular outflow obstruction who were undergoing cardiac catheterisation. There was satisfactory correlation with measured systolic pressure gradients in most patients with isolated pulmonary valve stenosis, aortic stenosis or aortic coarctation. Important under-estimation of gradients, however, occurred frequently in patients with more complex lesions. In many of these, the stenotic jet was posteriorly located or obstruction to flow occurred at more than one level. Continuous wave Doppler assessment of outflow tract gradients should be interpreted with caution in complex congenital heart lesions. A low predicted gradient should not be relied upon in isolation for clinical decision making. On the other hand, demonstration of a large gradient by continuous wave Doppler ultrasound provides additional information which may obviate the need for invasive investigation.

Repair of Aortic Coarctation in the First Three Months of Life: Immediate and Long-Term Results

The optimum surgical procedure for treatment of coarctation of the aorta in the neonatal period remains controversial. To assess immediate and long-term results of using primarily the subclavian flap angioplasty procedure (SFA), we reviewed our initial 5-year experience. The average follow-up was 6 years. From 1977 to 1981, 25 infants under 3 months of age (1 to 86 days, mean 21) required emergency surgery for repair of coarctation of the aorta. Three groups of patients were identified. Group I consisted of 10 patients with or without patent ductus arteriosus. In group II, 10 patients had coarctation associated with one or multiple ventricular septal defects (VSDs) without other congenital defects. In group III, 5 patients had coarctation associated with more complex congenital heart lesions. Twenty-three SFAs and two patch aortoplasties were performed. No patient with isolated VSD was banded. All patients except one in group III with an associated atrioventricular canal survived initial hospitalizations. Four late deaths occurred, all in patients with associated complex heart defects. There were three recurrent coarctations requiring surgery or balloon angioplasty (12%)—one in each group, with a total rate of 0.77 recurrences per 100 patient-months. SFA for coarctation in the neonatal period is a safe and effective operation with a low initial mortality (4%, 0-19%, 70% confidence limits) well tolerated in this group of ill patients. Long-term outcome is primarily related to the presence of associated complex congenital defects. Infants with VSD associated with coarctation did not require pulmonary artery banding unless primary intracardiac repair was not feasible. The recurrence rate is acceptably low, and recurrences may be effectively treated in certain cases with balloon angioplasty.

Transseptal left heart catheterization: experience with a new technique in 520 pediatric and adult patients.

A new technique for transseptal left heart catheterization utilizing the Transseptal Introducer Set in 520 patients is described in detail. The age range of these patients was 3 months to 40 years with 30% of the patients less than 2 years old and 28% less than 10 kilograms in weight. The safety and advantages of the technique are emphasized. The introducer set and new technique make transseptal left heart catheterization a safe, versatile, and dependable approach to all areas of the left heart, particularly in infants and children with complex congenital heart lesions.

Avoiding compression of extracardiac valved conduits.

Extracardiac valved conduits are routinely employed in the correction of complex congenital heart lesions. Compression of a conduit and/or of the heart may present serious operative and postoperative complications. We believe its occurrence can be minimized and hemodynamic results improved by adhering to simple surgical techniques. It is important to select a conduit which will produce minimal resistance to flow. Partial or subtotal thymectomy may increase the mediastinal space to accommodate the conduit. The conduit should be cut into the appropriate shape and should be placed in such a way that it does not cross the midline. Finally, the posterior pericardium may be opened to allow the heart to move posteriorly and to rotate to the left, thus moving the conduit away from the sternum.

Continuous Enteral Feedings

Eleven infants with complex congenital heart lesions were given continuous enteral infusions after failure to gain weight adequately despite use of hypercaloric formulas and nutritional supplementation. Formulas used before institution of enteral feedings were continued. Dietary and caloric intake and weight measurements were obtained at weekly or monthly intervals. Both mean daily caloric intake and mean daily weight gain were greater after initiation of continuous enteral feedings. Rate of weight gain improved in all 11 children after institution of enteral feedings. Continuous enteral feeding increases weight gain in babies with complex congenital heart disease, allowing earlier and safer surgical intervention.

Atrial repairs of infant complex congenital heart lesions: K. Turley, J. M. Wilson, and P. A. Ebert. Arch Surg 115: 1332–1337, (November), 1980

Atrial repairs of infant complex congenital heart lesions: K. Turley, J. M. Wilson, and P. A. Ebert. Arch Surg 115: 1332–1337, (November), 1980

Atrial repairs of infant complex congenital heart lesions. Emphasis on the first 3 months of life.

From 1975 to 1980, 106 infants in the first year of life with complex cardiac anomalies underwent isolated intra-atrial repair; 70 were in the first 3 months of life. Of these 70, 39 were 1 to 3 months old, 15 were 1 week to 1 month old, and 16 were less than 1 week old. The median age was 70 days, the range, 1 to 360 days. These infants included 74 with transposition of the great arteries, 28 with total anomalous pulmonary venous connection, and four with complex lesions (cor triatriatum and combined total anomalous pulmonary venous connection and anomalous systemic connection). Overall survival was 95%; for those in the first 3 months of life, 96%; for those in the first month, 94%; and for those in the first week, 100%. Age was not a determinant of survival. Attempts to delay surgical intervention once symptoms appear only results in clinical deterioration and interim mortality.

Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children.

Determination of atrial situs is of cardinal importance in the analysis of complex congenital heart lesions, and is best predicted from bronchial situs. Previous methods for assessing bronchial morphology, however, are unsuited to the very young patient. To assess bronchial morphology, 100 consecutive patients under 18 months of age (medium 57.5 days) with suspected congenital heart disease were studied by high kilovoltage filtered beam radiographs, before cardiac catheterisation. This low radiation dose technique clearly defined bronchial anatomy in 95 patients. The lengths of the left and right main bronchi were compared and 10 cases (10%) had a ratio less than 1.5 suggesting bronchial isomerism. Discriminant function analysis based on tracheal width and bronchial length enabled clear distinction of right from left bronchi. Four patients had bilateral right and six had bilateral left bronchi. Four of these 10 cases died and had necropsy confirmation of the radiological diagnosis. Practical prediction about cardiac anatomy, particularly the systemic and pulmonary venous return, may be made when bronchial morphology is known.

Advances in Cardiovascular Surgery

This small monograph probably represents the most valuable statement available today of the problems of cardiovascular surgery. Although describing himself in the foreword as generally unenthusiastic about books with multiple authors, Dr. Kirklin has chosen well and has brought his own editorial expertise in precise, lucid, orderly, and uncluttered presentation to bear on the contributions of 22 other leading contemporary pioneers in cardiovascular surgery. Several chapters describing management of complex congenital heart lesions are written conjointly by authoritative cardiologists and surgeons, and provide perspective and detail that might otherwise have been lacking. This monograph is not an exhaustive review of the literature. Authors present their own approaches and methods with adequate but not belabored supportive, descriptive, and comparative statistical data. Bibliographies accompanying each chapter are useful, for the most part brief, and provide a good balance between classic and current reference sources. Repetition has been avoided by careful editing. Illustrations,

Left retrograde cardioangiography: its diagnostic value in acquired and congenital heart disease.

Recent advances in cardiac surgery have made necessary the development of more accurate diagnostic tests for certain acquired and complex congenital heart lesions. We have found left retrograde cardioangiography the most reliable means of diagnosis for both aortic and mitral valvular disease. It has proved useful also in certain cases of congenital heart disease, particularly intracardiac and extracardiac left-to-right shunts. In this paper details of the technic employed at the University of Minnesota Hospitals will be given. In addition, the observations will be correlated with surgical data for patients who have been operated upon to date. Information concerning the relative diagnostic accuracy of conventional roentgenography will be included. History In 1933 Reboul and Racine (1) reported a technic for direct needle puncture of the left ventricle and injection of contrast material. In 1936 Nuvoli (2) opacified an aneurysm of the ascending aorta in a patient by this procedure. Ponsdomenech and Nunez (3...