[ON THE DIAGNOSTIC DIFFICULTIES OF THE TRANSPOSITION OF THE PULMONARY VEIN].
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Angiocardiography↗
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[TRICUSPID ATRESIA. (REVIEW OF 7 CASES)].
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Child↗
Bronchial compression due to stent placement in pulmonary artery in a child with congenital heart disease.
Congenital heart disease, such as transposition of the great vessels (TGV), requires surgical procedures which can lead to important complications. We report on a case of bronchial obstruction following placement of a pulmonary artery stent in a 4-year-old boy who had undergone a Rastelli procedure to correct TGV, ventricular septal defect and pulmonary stenosis. There are many complications that can arise as a consequence of intravascular stents in heart surgery, as well as many causes of bronchial compression. However we have not found any report which describes bronchial compression as a direct consequence of endovascular stent.
Bronchial Diseases↗
Should cardiac transplantation for congenital heart disease be delayed until adult age?
The number of pediatric heart transplantations for complex congenital heart disease has increased over the last years, but little experience has been reported in adolescent and adult populations. Between 1987 and 1992, 14 patients (mean age 33.2 years, range 14 to 51 years) were transplanted in our institution because of structural congenital heart disease (n = 9) or other rare disorders of the endomyocardial morphogenesis (n = 5). The main diagnosis included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypoplasia, double outlet right ventricle with transposition, left ventricular sinusoidal malformation and right ventricular dysplasia. In several cases there were additional intracardiac malformations, including ventricular septum defect, atrial septum defect as well as different forms of pulmonary stenosis. Seven patients had undergone one or more palliative repairs that consisted of modified Blalock-Taussig shunts, Glenn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atrioseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor cardiectomy was modified in order to include complete inflow and outflow tissue in the explant and transplantation could be performed without prosthetic material in all patients; deep hypothermic cardiac arrest was never necessary in this series. There was no early or late mortality after a mean follow-up of 37 months (range 4 to 74 months); postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results in all patients. Adolescent and adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Etiologic relations among categories of congenital heart malformations.
Pairs of siblings with congenital heart malformations of different types were analyzed for evidence of nonrandom association of defects within families that might suggest a genetic predisposition common to two or more kinds of malformations. An excess of pairs was noted for tetralogy of Fallot and pulmonary stenosis, tetralogy of Fallot and transposition of the great vessels, and tetralogy of Fallot and ventricular septal defect, thus suggesting that there may be a developmental relation between these lesions. This finding is supported by a recent study in the Keeshond dog demonstrating a genetic predisposition common to tetralogy of Fallot, pulmonary stenosis and ventricular septal defect. Thus the method does seem capable of revealing etiologic relations, probably genetic, among different types of cardiac lesions. Data on risks of recurrence for siblings of children with these defects will now have to be refined to take into account the possible recurrence of related lesions.
Child↗
Successful pregnancy in a woman with cyanotic congenital heart disease after a palliative pulmonary-systemic shunt. A case report.
A 20-year-old woman with cyanotic congenital heart disease composed of corrected transposition of the great vessels, severe pulmonic stenosis, atresia of the left pulmonary artery and a large ventricular septal defect, had a successful pregnancy following a pulmonary-systemic shunt (Blalock-Taussig). The hemoglobin decreased from 21 to 16 g/dL following the operation. The antepartum course was complicated by intrauterine growth retardation and pregnancy-induced hypertension. A normal fetal nonstress test and biophysical profile permitted continuation of the pregnancy until 38 weeks' gestation, with delivery of a healthy infant.
Abnormalities, Multiple↗
Migration and torsions of the conotruncus in the chick embryo heart: observational evidence and conclusions drawn from experimental intervention.
It seems difficult to escape the conclusion that conotruncal migration and torsions do occur: The amplitude, direction and timing of these movements can be accurately tracked and they can be experimentally arrested. The movements have a significant function: Associated with the partitionings, they regulate the outlet ventricular distribution at two levels, permitting an adjustment between the ampullae and conus (a function of migration) and between the proximal and distal conal segments (via torsions). Their defects result in malalignments--double outlet right ventricle and transposition of the great vessels, respectively. The mechanism of the migration is linked to the differential growth of ampullae; the right ampulla and certain areas of this ampulla appear as dominant during the embryonic period. The conus is carried by the right ampulla and passively undergoes migration. The mechanism of the torsions is not entirely clear. The proximal torsion appears to be linked to a myocardium--jelly dissociation; the distal torsion seems to be linked to an oriented myocardial growth of its wall; the torsion of the truncus is evidently passive, occurring as a consequence of the conus distal torsion.
Animals↗
Ascending aorta-right pulmonary artery shunt.
Seventy-seven patients with ascending aorta-right pulmonary artery shunt were reviewed; 48 had tetralogy of Fallot, 9 had pulmonary atresia, 11 had transposition of the great vessels with pulmonary stenosis, 4 had tricuspid atresia, and 5 had miscellaneous complex lesions. Their ages ranged from one day to 13 years. The over-all mortality rate was 17.8 per cent. Ten patients underwent total repair and takedown of the shunt with no deaths. The problems of increased pulmonary flow, pulmonary hypertension, preferential flow to one lung, kinking and distortion of the pulmonary artery, and the technical difficulties at the time of takedown were reviewed and discussed.
Aorta↗
[Hemifacial microsomia and cardiac malformations. Apropos of 2 cases].
The authors report two cases of infants with complex heart disease comprising transposition of the great vessels, and a major unilateral facial anomaly involving the ear and the mandible, conforming to the description of hemifacial microsomia. The facial anomalies seen are defined and placed in the classification of known syndromes. The incidence of heart disease associated with facial microsomia is about 20% and in 2/3 of cases it is a ventricular septal defect or a tetralogy of Fallot. An analysis of aetiological factors shows that syndromes of the first two branchial arches are usually sporadic and that they may be the result of intra-uterine disease. The mechanism of the facial lesions is probably vascular.
Abnormalities, Multiple↗
Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease.
We report our initial experience with three patients who received heart-lung transplants. The primary immunosuppressive agent used was cyclosporin A, although conventional drugs were also administered. In the first patient, a 45-year-old woman with primary pulmonary hypertension, acute rejection of the transplant was diagnosed 10 and 25 days after surgery but was treated successfully; this patient still had normal exercise tolerance 10 months late. The second patient, a 30-year-old man, underwent transplantation for Eisenmenger's syndrome due to atrial and ventricular septal defects. His graft was not rejected, and his condition was markedly improved eight months after surgery. The third patient, a 29-year-old woman with transposition of the great vessels and associated defects, died four days postoperatively of renal, hepatic, and pulmonary complications. We attribute our success to experience with heart-lung transplantation in primates, to the use of cyclosporin A, and to the anatomic and physiologic advantages of combined heart-lung replacement. We hope that such transplants may ultimately provide an improved outlook for selected terminally ill patients with pulmonary vascular disease and certain other intractable cardiopulmonary disorders.
Adult↗
Lack of connection between the atria and the peripheral conduction system in a case of corrected transposition with congenital atrioventricular block.
This is the first documented histologic study of the heart of a patient with corrected transposition of the great vessels and congenital atrioventricular (A-V) block with no connection between the atria and an anterior type of peripheral conduction system. Musculature in the superior (anterior) walls of both atria was absent, as was the anterior A-V node. The peripheral conduction system began with the bundle of His. In place of the absent atrial musculature, fibrosis and calcification were present. The relation of laboratory evidence of connective tissue dyscrasia in the mother to the congenital A-V block in the child is discussed.
Adult↗
Esmolol (Brevibloc) to assess dynamic subpulmonary stenosis in a child after repair of complete transposition: a case report.
Esmolol (Brevibloc), a new, ultra-short acting, cardioselective beta-adrenergic blocking agent with half-life of 9.2 min following i.v. administration was given to a 4-year-old child with known dynamic and fixed sub-pulmonary stenosis post Senning repair for complete transposition of the great vessels. The left ventricular systolic pressure increased from 48 to 100 mmHg, heart rate showed an increase from 65 to 140 bpm, the right femoral arterial pressures decreased from 115/58 to 77/35 mmHg following an infusion of Isoprel. Infusion of esmolol partially relieved the dynamic sub-pulmonary stenosis. There were no adverse effects and esmolol was tolerated well by the child. Esmolol might thus play a role in the pediatric catheterization laboratory during investigational procedures, electrophysiological studies and in the control of rapid supraventricular tachycardia, especially in adolescents with WPW pre-excitation. Esmolol would also be beneficial in emergency treatment of epinephrine or isoproterenol overdosage.
Adrenergic beta-Antagonists↗
Corrected transposition with severe intracardiac deformities with Wolff-Parkinson-White syndrome in a child. Electrophysiologic investigation and surgical correction.
This is a report of a 10-year-old child who underwent surgery for complex congenital heart disease consisting of corrected transposition of the great vessels, ventricular septal defect, patent ductus arterisus, severe left-sided atrioventricular (AV) valve insuffieicney (Ebstein's deformity) and Wolff-Parkinson-White syndrome. The site of his accessory AV connection was localized preoperatively at a left anterolateral site by isopotential body surface maps and by intracardiac electrophysiologic studies. He successfully underwent surgery for closure of the ventricular septal defect, ligation of the patent ductus arteriosus, replacement of the left-sided AV valve, and interruption of the accessory AV pathway. Unavoidable complete AV block acquired at surgery required subsequent permanent pacemaker therapy.
Cardiac Catheterization↗
Ventricular paired pacing to control rapid ventricular heart rate following open heart surgery. Observations on ectopic automaticity. Report of a case in a four-month-old patient.
An unusual case is presented in which an atrioventricular (A-V) junctional tachycardia at a rate of 285 beats/min developed in the immediate postoperative period following surgical repair (a Mustard procedure) of transposition of the great vessels in a four-month-old infant. With that heart rate the systolic blood pressure bacame 35-40 mm Hg and urinary output ceased. Ventricular paired pacing was employed successfully to halve the mechanically effective ventricular rate. This resulted in a clinically effective blood pressure and return of normal urinary output. The clinical course of the A-V junctional tachycardia, plus its response to several interventions, suggested that the mechanism of the A-V junctional tachycardia was automatic rather than re-entrant. The study demonstrates that ventricular paired pacing for the control of cardiac arrhythmias is a useful clinical technique in selected cases.
Atrioventricular Node↗
Transesophageal echocardiographic guidance of balloon atrial septostomy.
Two newborn infants with transposition of the great vessels and an intact ventricular septum were cyanotic when they underwent bedside balloon atrial septostomy in the neonatal intensive care unit. The procedure was conducted under direct transesophageal two-dimensional echocardiographic guidance. Success of the procedure was evaluated with color flow Doppler and the two-dimensional assessment of the septostomy. This procedure has advantages over catheterization and its attendant fluoroscopy, which usually are performed in the catheterization laboratory, expose the patient to radiation, and necessitate transportation of a patient who is hemodynamically unstable to an often distant catheterization laboratory. Transesophageal echocardiography has several advantages over transthoracic echocardiography in aiding this procedure. It provides an optimal view of the atrial septum, while keeping the chest exposed for observation of respiratory effort and for improved thermoregulation.
Catheterization↗
Congenitally corrected transposition in adults with left atrioventricular valve incompetence.
Congenitally corrected transposition of the great vessels (CCT) is usually detected in childhood owing to the presence of associated cardiac malformations. If such malformations are absent, the patient may remain asymptomatic until adulthood; such persons usually present with left atrioventricular valve incompetence and conduction disturbances. The chest radiograph may be the first clue to the diagnosis, demonstrating the typical cardiovascular silhouette produced by the convex left ascending aorta. The authors examined seven cases of CCT manifested in adulthood and found that in four of them the diagnosis was not apparent from the plain image because the ascending aorta instead followed a concave course. Hallmarks of this phenomenon are discussed, and two radiological signs are presented: the double mediastinal stripe and the straight left lower cardiac border.
Adult↗
Mesangiolysis in the glomerular lesion of cyanotic congenital heart disease.
Mesangiolysis associated with glomerulomegaly was observed in an autopsy case of an 11-year-old boy with cyanotic congenital heart disease, i.e. complete transposition of the great vessels with large VSD. Microscopical examination of the kidney revealed marked enlargement of glomeruli with mesangiolysis mainly found in the midzonal cortex involving 16 to 20% of all glomeruli. The glomerular basement membrane in the mesangiolytic lesion showed splitting, between which red blood cells were occasionally encountered. By reconstruction study with serial sectioning, the mesangiolysis was observed to be obviously focal and segmental. Immunofluorescent study revealed no positive results for IgG, IgA, IgM, C3, and fibrinogen/fibrin. By electron microscopic study on the post formalin fixed specimens, no remarkable dense deposits were found in any structural components of the glomeruli. It was assumed that the mesangiolysis might have been provoked by some circulatory disturbances, which superimposed on the preexisting glomerular lesion of cyanotic congenital heart disease in the terminal stage of the clinical course.
Child↗
The Blalock-Hanlon procedure. Simple transposition of the great arteries.
Between 1959 and 1975 at St. Louis University Medical Center, 71 patients underwent surgery with the Blalock-Hanlon technique. Thirty-nine had simple transposition of the great vessels. The mean age at the time of operation was 3.4 weeks. Sixty-four percent were less than 1 month of age. Eighty-five percent survived the operation. In 11 the Blalock-Hanlon procedure was performed after failure of ballon septostomy. Arterial saturation was increased from a mean of 47% to 73%. There were three late deaths prior to Mustard repair (intra-atrial baffle procedure). Sixteeen patients underwent Mustard repair at a mean age of 4 years and a mean follow-up of 2 1/2 years. There were three deaths after surgery and three late deaths after the Mustard procedure. The Blalock-Hanlon procedure achieves prolonged palliation, avoiding an emergency Mustard procedure in infancy with its risk of late vena caval obstruction.
Cardiac Surgical Procedures↗