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Prostaglandin E1 is commonly used in the management of cyanotic congenital heart disease. While cutaneous flushing and peripheral edema are well recognized side effects of prostaglandin E1 therapy, other cutaneous effects have not been described in the dermatologic literature. We report a neonate with transposition of the great vessels who developed urticaria during treatment with prostaglandin E1.
In 3 patients with a congenital aneurysm of the membranous portion of the ventricular septum diagnosis could be made by angiography. One of these patients was clinically asymptomatic, while the two other patients were transferred for surgery because of right ventricular outflow tract obstruction or intracardiac left-to-right shunt. These both patients underwent successful open heart corrections. Aneurysms with a different intracardiac localization could be found. One of the cases represents an unusual association with corrected transposition of the great vessels.
Coronary artery stenosis (CAS) and coronary ostial stenosis (COS) are potentially life-threatening conditions. The echocardiographic diagnosis of CAS and COS in children has not been described. We report on the transthoracic echocardiography (TTE) findings of CAS and COS in children. Six patients, aged 1 week to 12 years, with clinically confirmed COS (n = 5) and CAS (n = l) were diagnosed by TTE. Their echocardiographic findings were compared with 26 healthy control subjects of a similar age range. Left COS was associated with an aberrant left coronary artery (CA) from the contralateral aortic sinus (n = 2), an intramural left CA with d-transposition of the great vessels (n = l), and supravalvular aortic stenosis (n = l). Right COS was present in a patient with aortic valvular stenosis. Acquired left main CAS was diagnosed in the sixth patient 3 years after orthotopic heart transplantation. Coronary ostial stenosis was recognized when a color flow acceleration signal was present proximal to and extending into the coronary ostium (CO). Coronary artery stenosis was detected when a coarctated color flow stream was present within the stenosed CA segment with turbulent distal flow. These findings were not detected in the control cohort who demonstrated laminar CA and CO flow signals. All patients had increased spectral velocity in the CA distal to the stenosed segment (patients = 50 +/- 5 cm/s, controls = 24 +/- 6 cm/sec; P <.01). Delayed peak diastolic velocity seemed to indicate severe stenosis. We conclude that (1) CO acceleration signals and turbulent coarctated CA flow signals are abnormal findings in TTE coronary Doppler assessment. They indicate COS and CAS, respectively. (2) Knowledge of the normal TTE CA flow velocity patterns is essential so that abnormal velocity signals such as seen with CAS and COS can be recognized and a timely diagnosis made.
Exercise-induced collapse and sudden death are unusual in childhood. For this reason, a study was undertaken of a series of 12 cases of sudden death in childhood occurring during physical exertion associated with sporting activities. The age range was 7 to 16 years (mean 12.3 years, M:F ratio 5:1). Deaths resulted from trauma associated with the sporting activity, from an idiosyncratic response to exertion, or from exacerbation of a known underlying disease. Trauma was directly fatal (n = 4: vascular trauma in 1, head injury in 2, drowning in 1), exacerbated an underlying medical condition (n = 1: hypertrophic obstructive cardiomyopathy), or followed collapse from underlying organic disease (n = 1: drowning in epilepsy). Deaths after exertion occurred when there was an unexpected response to underlying occult disease (n = 4: aortic stenosis in 1, cerebral arteriovenous malformation in 1, hypertrophic obstructive cardiomyopathy in 1, coronary atherosclerosis in 1) or to preexisting known disease (n = 2: surgically corrected transposition of the great vessels in 1, asthma in 1). The fatal episodes often resulted from a complex interplay of a variety of factors, including physical exertion, possible trauma, and underlying organic disease. Testing of other family members may be indicated in cases where a rare, possibly familial, disease is found. Evaluation of cases required descriptions of activities before death, information from the medical history of the deceased, and detailed findings from the autopsy.
In reviewing selected publications on congenital heart disease management for 1990 and 1991, some topics have been chosen for discussion, including isolated patent ductus arteriosus, coarctation of the aorta, critical aortic stenosis in early infancy, hypoplastic left-heart syndrome, pulmonary artery banding, Fontan procedures, pulmonary atresia and intact ventricular septum, Ebstein's anomaly, tetralogy of Fallot, transposition of the great vessels, and others.
The management of congenital heart disease is in a state of evolution, with earlier surgical and/or catheter interventions dominating clinical decision-making. Recent advances in interventional cardiac catheterization techniques, as well as continuing advances in the surgical management of complex congenital defects, continue to be the focus of attention of cardiologists and surgeons. The majority of the papers reviewed document mid- and long-term results of specific operative procedures for and address the appropriate role of cardiac catheterization techniques in the management of transposition of the great vessels, tetralogy of Fallot, total anomalous pulmonary venous connection, truncus arteriosus, pulmonary atresia with intact ventricular septum, and the univentricular heart. Early definitive intervention has become the standard of care for almost all defects reviewed.
The authors evaluated the clinical applications of Tc-99m methoxy isobutyl isonitrile (MIBI) in the simultaneous assessment of ventricular function and myocardial perfusion in patients with congenital heart disease. Global ventricular function was assessed by first-pass ECG gated study. Myocardial perfusion was evaluated on images performed 1 hour after the injection of the tracer. Regional wall motion and systolic thickening were assessed by gated study acquired after the perfusion study. Two young patients were studied after a surgical Glenn procedure. The first patient, with a history of transposition of the great vessels and univentricular heart, had an ejection fraction of 44%. The left ventricle was dilated and the right ventricle was not appreciable. The septal and inferoapical regions showed reduced perfusion and reduced systolic thickening. The second patient, with a history of pulmonary atresia, septal defect and left ventricular hypoplasia, had a right ventricular ejection fraction of 37%. Regional wall motion, systolic thickening, and myocardial perfusion were normal. The right ventricle was hypertrophic and larger than the left ventricle. Thus, a single injection of Tc-99m MIBI allows noninvasive simultaneous assessment of global and regional ventricular function and myocardial perfusion in young patients with complicated congenital heart disease.
Developmental growth toward, and fusion at, the midline of the paired first branchial arches results in normal mandibular integrity. Failure of this process is the cause of midline mandibular clefts and associated clefts of the lower lip and anterior tongue. A case is presented with these findings, complicated by the presence of a single ventricle and transposition of the great vessels. A successful one-stage repair of the clefts of the lower lip, tongue, and mandible was performed.
Nicardipine is the first intravenously administered dihydropyridine calcium channel blocker. Its primary physiologic action includes vasodilatation with limited effects on the inotropic and dromotropic function of the myocardium. Several previous reports document its use in adult patients for pharmacologic control of blood pressure. The current report describes the use of nicardipine to control mean arterial pressure (MAP) in nine infants and children after cardiothoracic surgical procedures. The patients ranged in age from 6 days to 9 years (mean, 3.3 +/- 4.1 years) and in weight from 4.1 to 49 kg (mean, 15.3 +/- 14.4). The surgical procedures included aortic coarctation repair (three), repair of tetralogy of Fallot (two), arterial switch for transposition of the great vessels (two), pulmonary valvotomy (one), and aortic valvotomy (one). The target systolic blood pressure was 90 mm Hg in patients younger than 4 years of age and < or = 110 mm Hg in patients 5 years of age or older. The nicardipine infusion was started at 5 microg/kg/min in all patients. The target blood pressure was achieved within 15 minutes in eight of nine patients. One patient required an initial infusion rate of 10 microg/kg/min to achieve the target blood pressure. The maintenance infusion rate varied from 2.5 to 5.5 mcg/k/min (mean 3.0 +/- 1.1). The duration of the infusion varied from 30 to 42 hours (mean, 37.4 +/- 4.2). In the nine patients, nicardipine was infused for a total of 337 hours. No adverse effects such as excessive hypotension were noted. Nicardipine is an effective agent for controlling MAP after cardiothoracic surgical procedures in infants and children.
Currently, there are 1 million people in the United States over the age of 20 with congenital heart disease. These adult congenital heart patients can "slip through the cracks of our medical system" and many are too old to be cared for in most pediatric institutions by pediatric cardiologists, and, unfortunately, most adult cardiologists are not trained in congenital heart disease. Therefore, it is important to understand what the common lesions are in adult congenital heart disease and how they should be managed. Acyanotic congenital heart disease in the adult population primarily involves left-to-right shunts, such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and obstructive lesions such as aortic coarctation of the aorta. The most common form of cyanotic congenital heart disease in adults is tetralogy of Fallot. Other complex conditions seen in adults include univentricular hearts, Ebstein's anomaly of the tricuspid valve, and corrected transposition of the great vessels. Most patients with congenital heart disease will need to undergo surgery, catheterization, or catheterization intervention. Results are excellent in the adult population. Long-term follow-up is needed for any adult congenital heart patient receiving care in institutions that are well organized and well equipped, as we learn more about the natural and unnatural history of these conditions.
We describe a case of a 50-year-old woman with congenitally corrected transposition of the great vessels, in whom severe left-sided tricuspid (systemic atrioventricular) valve insufficiency was the only associated anomaly. The tricuspid valve was dysplastic and abnormally oriented toward the interventricular septum, without the downward displacement of Ebstein's anomaly. The mechanism of atrioventricular regurgitation was unusual in that it consisted of the rupture of chordae tendineae of both the anterior and septal leaflets. The left-sided tricuspid valve was replaced with a St. Jude prosthesis and the postoperative course was uneventful.
In the family presented here the first child had asplenia syndrome with cor biloculare' transposition of the great vessels, pulmonary stenosis, and anomalous pulmonary venous drainage. Another sib had situs inversus with polysplenia syndrome, including very similar cardiovascular defects and biliary atresia. The possibility that these two syndromes, namely asplenia and polysplenia, are different manifestations of a similar defect in the normal asymmetrical development of internal organs is discussed.
The reliability of prenatal ultrasound (US) for the detection of structural heart defects was investigated in a consecutive population of patients referred for prenatal US. Twenty-eight of 49 fetuses (57%) shown to have one or more heart defects with physical examination or autopsy were found to have congenital heart disease with prenatal US. There were 66 cardiac defects in 49 fetuses, 33 of which were entirely or partly identified (50%). Defects correctly diagnosed included hypoplasia of either ventricle, common atrioventricular canal, tetralogy of Fallot, transposition of the great vessels, and Ebstein anomaly. Defects rarely diagnosed correctly included semilunar valve stenosis, total anomalous pulmonary venous drainage, coarctation of the aorta, pulmonic atresia, atrioseptal defect, and ventricular septal defect. Many types of heart defects can be diagnosed accurately with prenatal US in a general population of patients not at high risk for congenital heart disease. Some lesions, however, were difficult to detect.
BACKGROUND: Results of staged palliation for hypoplastic left heart syndrome (HLHS) have improved in recent years; however, certain risk factors have been associated with decreased survival rates. METHODS AND RESULTS: We retrospectively reviewed the medical records of 67 patients weighing </=2.5 kg undergoing the first stage of reconstructive surgery at our institution between January 1, 1990, and December 31, 1997. HLHS was present in 45 patients, complex double-outlet right ventricle in 10, unbalanced AV canal in 5, tricuspid atresia with transposition of the great vessels in 4, and other diagnoses in 3. Mean age at surgery was 10.1+/-10.7 days (median, 8 days), and mean weight was 2.2+/-0.3 kg (median, 2.2 kg). Fourteen patients weighed </=2.0 kg, and 2 patients weighed </=1.5 kg. Early mortality (death within 30 days or before hospital discharge) was 51% (34 of 67). No patient, procedural, or time-related variables correlated with increased mortality. However, there was a trend toward increased mortality with increased cardiopulmonary bypass time (P=0.076) and decreased preoperative ventricular performance (P=0.139). CONCLUSIONS: These findings suggest that low weight alone in a patient with HLHS or an anatomic variant should not be considered a contraindication to staged reconstructive surgery.
BACKGROUND: Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. METHODS AND RESULTS: We reviewed the pretransplantation and posttransplantation courses of 24 patients >18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d-transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years. No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). CONCLUSIONS: Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.
Balloon dilation angioplasty was attempted in 10 infants and children with severe congenital or operative "venous" obstructions. In five children the obstructions were "vena caval" and followed repair for transposition of the great vessels (four patients) or orthotopic liver transplantation (one patient). In the four patients with fixed vena caval or baffle obstructions, balloon angioplasty was successful in relieving the obstruction, decreasing the average gradient (16.0 to 4.5 mm Hg), and increasing the average diameter (3.0 to 8.9 mm) of the obstructed site. One child died 1 week later from an unrelated cerebral hemorrhage; the three survivors have had persistent clinical and angiographic improvement. The fifth child had severe systolic narrowing of the superior baffle limb caused by marked tricuspid regurgitation, which ballooned the superior limb of the baffle against the atrial roof. Angioplasty was unsuccessful in relieving this type of obstruction, which resolved with tricuspid valve replacement. Of the five infants with obstructed pulmonary veins, three had congenitally narrowed vessels associated with total anomalous pulmonary venous connection, one had acquired stenosis, and one had postoperative obstruction after repair of a mixed type of total anomalous pulmonary venous connection. Dilation was unsuccessful in all five patients, but for different reasons: in the congenitally narrowed veins, the waist in the balloon could not be eliminated, even with high dilating pressures; in the infant with acquired stenosis, the vein stretched but did not tear at low dilating pressures; and in the postoperative obstruction, angioplasty increased the diameter of the obstruction but did not increase flow to the affected lung.(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: Although long-term prostacyclin (PGI2) has been shown to improve hemodynamics, quality of life, and survival in patients with primary pulmonary hypertension, its use in patients with pulmonary hypertension (PHT) and associated congenital heart defects (CHD) has not been evaluated. METHODS AND RESULTS: Twenty patients (15+/-14 years) with PHT and associated CHD (9 atrial septal defect, 7 ventricular septal defect, 4 transposition of the great vessels, 3 patient ductus arteriosus, 3 partial anomalous pulmonary venous return, and 1 aortopulmonary window) who failed conventional therapy (including digitalis; diuretics; oxygen; warfarin; calcium channel blockade, if indicated; and surgery, if operable) were treated with chronic PGI2. Eleven patients had previous cardiac surgery at a median age of 3 years (range, 5 days to 47 years). Eleven of 20 patients had residual systemic to pulmonary shunts. Hemodynamics, NYHA functional class, and exercise capacity were measured at baseline and after 1 year of PGI2 therapy. None of the patients acutely responded to PGI2 administration. Despite lack of an acute response, mean pulmonary artery pressure decreased 21% on chronic PGI2: 77+/-20 to 61+/-15 mm Hg (P<0.01, n=16). Cardiac index and pulmonary vascular resistance also improved on long-term PGI2: 3. 5+/-2.0 to 5.9+/-2.7 L. min-1. m-2 (P<0.01, n=16), and 25+/-13 to 12+/-7 U.m2 (P<0.01, n=16), respectively. NYHA functional class improved from 3.2+/-0.7 to 2.0+/-0.9 (P<0.0001, n=19). Exercise capacity increased from 408+/-149 to 460+/-99 m (P=0.13, n=14) on long-term PGI2. CONCLUSIONS: Chronic PGI2 improves hemodynamics and quality of life in patients with PHT and associated CHD who fail conventional therapy. As previously demonstrated in patients with primary pulmonary hypertension, long-term PGI2 may have an important role in the treatment of patients with PHT and associated CHD.
We reviewed autopsies in 50 consecutive cases of congenital heart disease between 1980 and 1988. Autopsy results were compared to premortem clinical diagnosis. The patients ranged in age from two days to 66.5 years with a mean age of 6.42 years; 68% were less than one year of age. The most common diagnoses were hypoplastic left heart syndrome in 16 (32%) and ventricular septal defect in five (10%). Other common diagnoses included coarctation of the aorta, hypoplastic right heart syndrome, tetralogy of Fallot, transposition of the great vessels and tricuspid atresia. No patient had a missed diagnosis that would have changed survival (Class 1). Four (8%) of the patients had one missed major diagnosis that did not change their survival because they were already being symptomatically treated or because no clinical treatment was available (Class 2). The majority of autopsy derived data were missed minor diagnoses that were related [14 (28%)-Class 3] or unrelated [12 (24%) - Class 4] to the terminal disease process. However, despite the small percentage of autopsy findings that would have directly affected survival, the autopsy provided important additional clinical information in 30 (60%) patients. This information could be used to correct management errors and to develop protocols for prophylaxis against known complications. The pediatric autopsy can provide useful clinical data that may be a guide to altering clinical therapy and is essential in providing genetic counseling.