Prenatally diagnosed hypoplastic left heart syndrome--outcomes after postnatal surgery.

OBJECTIVE: To identify prenatally diagnosed cases of hypoplastic left heart syndrome (HLHS) and then to determine postnatal outcomes after surgical interventions. METHODS: An ultrasound and pediatric cardiology database was used to identify all fetuses diagnosed prenatally from 1991-1996 with HLHS. Fetal karyotypes were performed on cultured amniocytes. After diagnosis, parents were given several management options: pregnancy termination before 22 weeks, postnatal hospice care, or surgery using the Norwood procedure or cardiac transplantation. Ultrasound and echocardiography findings were later compared to karyotype results and postnatal outcome data. RESULTS: Fifteen fetuses with HLHS were identified. Two (16%) chromosome abnormalities and three (20%) structural defects were detected. Three mothers (20%) opted for pregnancy termination, two (13%) chose postnatal hospice care, and one aneuploid fetus had an intrauterine death. Nine parents (60%) chose surgery for their infants; however, one infant was not an appropriate surgical candidate due to a coexisting diaphragmatic hernia. Eight infants underwent surgery and two survived (25%). Of the four infants scheduled to undergo the Norwood procedure, one died preoperatively, two died intraoperatively, and one infant survived and is doing well at age 8 months. Of the four infants scheduled for cardiac transplantation, two died awaiting transplant and one died postoperatively. One infant survived cardiac transplantation but has microcephaly and developmental delay at age two. CONCLUSIONS: In prenatally diagnosed HLHS at our institution, the survival rate following surgery for infants felt to be the best candidates was only 25%.

Pulmonary vein stenosis after catheter ablation of atrial fibrillation.

BACKGROUND: This report describes the complication of pulmonary vein stenosis with resultant severe pulmonary hypertension that developed in 2 patients after successful catheter ablation of chronic atrial fibrillation. METHODS AND RESULTS: Three months after successful catheter ablation of atrial fibrillation, both patients developed progressive dyspnea and pulmonary hypertension. Both were found to have severe stenosis of all 4 pulmonary veins near the junction with the left atrium. Balloon dilation of the stenotic pulmonary veins was performed in these patients, with improvement in dyspnea and pulmonary hypertension. CONCLUSIONS: The complication of pulmonary vein stenosis is potentially life-threatening, and the application of radiofrequency current within the pulmonary veins with standard catheter technology should be avoided. This complication can be treated with balloon dilation, although the long-term course is unknown.

Etiology and retrieval of retained central venous catheter fragments within the heart and great vessels of infants and children.

BACKGROUND: The use of centrally positioned venous catheters plays an indispensable role in the care of infants and children. METHODS: Since 1992 the authors have seen nine patients who experienced fragmentation and migration of catheter fragments into the central circulation. The patients ranged in age from 6 days to 15 years. RESULTS: Sites of migration included pulmonary artery (five patients), superior vena cava (two patients), hepatic vein and innominate vein (one patient). The elapsed time from recognition of retained catheter fragments until retrieval ranged from a few hours to 6 weeks. CONCLUSION: All retained fragments were successfully removed during cardiac catheterization without complications.

Vegetation biopsy using transesophageal echocardiography guidance: a technique to aid in diagnosis of culture-negative endocarditis.

A method of obtaining a vegetation sample in a culture-negative endocarditis is described. A combination of fluoroscopy and transesophageal echocardiography was utilized to obtain the sample. The results positively influenced the diagnosis and treatment in this 16-yr-old male with complex congenital heart disease.

Fetal echocardiography in gravidas with historic risk factors for congenital heart disease.

All fetal echocardiograms performed at our institution between January 1, 1986, and June 1, 1991, were reviewed. The echocardiogram referral indication and results were verified for all women studied. Historic risk factors under consideration were pregestational diabetes, anticonvulsant or lithium ingestion in the first trimester, and a family history of congenital heart disease. Women with historic risk factors who had either a fetal abnormality on antenatal sonography or a known aneuploid fetus prior to the fetal echocardiograph were excluded. Of the 994 women who had a fetal echocardiogram performed during this study period, 486 (48.9%) were evaluated solely on the basis of a historic risk factor. Four of the 486 women (0.8%) were diagnosed as having a significant fetal structural cardiac malformation. Two of the four fetuses succumbed to obstetric complications. The remaining two infants were delivered at term and are doing well after postnatal cardiac surgery. In this select group of women with risk factors but no recognized fetal abnormality, the incidence of significant cardiac lesions was low. Although indications for fetal echocardiography must be evaluated on an individual basis, our data do not support a recommendation for the routine use of fetal echocardiography as a screening test in all women with historic risk factors.

Congenital heart disease in infants with Down's syndrome.

Medical records of 118 newborn infants with Down's syndrome were reviewed to document the types of congenital heart disease (CHD) in those having echocardiography. Of 102 infants having echocardiography, 49 (48%) had heart defects; 47 of these had trisomy 21 and 2 had unbalanced translocation karyotypes. Of the 53 (52%) who did not have heart defects, all had trisomy except 1 with a mosaic karyotype and 1 with a translocation karyotype. The most common heart malformation was an atrioventricular canal, followed in frequency by ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Benefits of echocardiography in such infants are early detection of CHD, with aggressive management to prevent future complications, and reassurance to parents if the infant does not have CHD.

The dimensions of the right ventricular outflow tract and pulmonary arteries in tetralogy of Fallot and pulmonary stenosis.

Studies were undertaken of the cineangiograms in 196 consecutive patients entering two institutions with tetralogy of Fallot and pulmonary stenosis, none of whom had previously undergone a surgical procedure. The median age of the patients at the time of the study was 5.9 months. The diameters of the right ventricular infundibulum, pulmonary trunk, and the entirety of the right and left pulmonary arteries were measured (in millimeters), corrected for magnification, and expressed in standard deviation units (Z-values). The median values of the cineangiographically determined diameters of the right ventricular infundibulum and pulmonary trunk were smaller than those of 95% of normal individuals. The median values throughout the right and left pulmonary arteries were within the range of normal. Those of the distal branches of both the right and left pulmonary arteries were similar to the mean values in normal individuals. However, great variability of the dimensions between individuals, and along the pathway in individuals, characterized patients with tetralogy of Fallot. Diffuse narrowing of the pathways both proximally and distally was uncommon. The relation between the diameters of the pulmonary "anulus" and of the distal pulmonary trunk and origin of the left pulmonary artery explained the difficulty of extending an enlarging patch into a wide area distally in some patients.

Management of prenatally detected nonlethal fetal anomalies: is a karyotype of benefit?

The pregnancy outcomes of 155 women who underwent an amniocentesis for a prenatal karyotype after being diagnosed by ultrasound as having one or more nonlethal structural anomalies are presented. Thirty-three (21%) patients were found to have an abnormal karyotype. Knowledge of the prenatal karyotype was useful in the subsequent management of these pregnancies. A pregnancy with a fetal anomaly diagnosed prior to 24 weeks was more likely to be terminated if an abnormal karyotype was also present. In women who were diagnosed as having a fetal anomaly with an abnormal karyotype at 24 weeks or later, only 3 of 13 (23%) infants survived the neonatal period. Knowledge of the karyotype results influenced decisions regarding the place, timing, and route of delivery in these fetuses. In 32 women, (21%) a karyotype was beneficial by avoiding maternal transport, cesarean delivery, and neonatal expenses at a Level III perinatal center.

Transesophageal echocardiography in aortopulmonary communications.

The assessment of congenital aortopulmonary communications by transthoracic echocardiography may be suboptimal, particularly postoperatively, due to limited acoustic windows. We performed intraoperative transesophageal echocardiograms in six patients with eight proven systemic-pulmonary communications. Diagnosis included truncus arteriosus (1), aortopulmonary window (1), Waterston anastomosis (3), central Gore-Tex shunt (1), and modified left Blalock-Taussig shunt (2). All communications were accurately demonstrated by transesophageal echocardiography (TEE). The transesophageal technique also provided an assessment of the size of the aortopulmonary communications and the proximal pulmonary arteries. In addition, the gradient across some of the communications could be accurately estimated utilizing the high pulse repetition frequency Doppler. On the other hand, only two of the seven aortopulmonary communications were detected by transthoracic echocardiography. Postoperatively, transesophageal imaging demonstrated unobstructed conduit in five of five patients who underwent conduit repair, as well as intact closure of aortopulmonary communications and concomitant closure of ventricular septal defects.

Transplacental transfer of zidovudine in the near-term pregnant baboon.

Approximately one third of infants born to human immunodeficiency virus type 1 seropositive mothers have evidence of infection or of acquired immunodeficiency syndrome by the age of 18 months. One fifth of infected infants also have died by age 18 months. This prevalence, combined with the demonstration that zidovudine (formerly azidothymidine) can decrease mortality and the frequency of opportunistic infections in patients with acquired immunodeficiency syndrome or acquired immunodeficiency syndrome--related complex, may lead to increasing use of azidothymidine in pregnancy despite a paucity of information regarding its pharmacokinetics. To further investigate the distribution of azidothymidine and its inactive metabolite 5'-glucuronide azidothymidine in the mother, fetus, and amniotic fluid, 12 near-term pregnant baboons were given oral azidothymidine (21 mg/kg/day in four divided doses every 6 hours, equivalent to the usual nonpregnant human dose of 1500 mg/day). Specimens of maternal blood, fetal arterial blood obtained by percutaneous umbilical cord blood sampling, and amniotic fluid were obtained after from one to 17 doses of azidothymidine. Azidothymidine levels were measured by radioimmunoassay with the INCSTAR commercial radioimmunoassay kit and using Escherichia coli beta-glucuronidase for determination of 5'-glucuronide azidothymidine levels. Paired analyses revealed significant concentration gradients between amniotic fluid, fetal serum, and maternal serum for both azidothymidine (p less than 0.019) and 5'-glucuronide azidothymidine (p less than 0.002). The amniotic fluid 5'-glucuronide azidothymidine level increased with increasing doses of azidothymidine despite the fact that the maternal azidothymidine and 5'-glucuronide azidothymidine concentrations were unchanged. This accumulation of amniotic fluid 5'-glucuronide azidothymidine may provide a functional drug reservoir and contribute to the higher fetal concentrations of the medication and its metabolite. Alternatively, the higher fetal levels may represent slower clearance in the fetus than in the mother. Further studies appear warranted with respect to possible adverse fetal effects, especially bone marrow suppression with prolonged and chronic exposure to azidothymidine.

Balloon dilation of miscellaneous lesions: results of Valvuloplasty and Angioplasty of Congenital Anomalies Registry.

Data on 111 lesions, in addition to pulmonary valve, aortic valve, branch pulmonary stenosis, native coarctation and recoarctation, were submitted to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Because there was a very heterogenous group of lesions both in types of lesions and techniques of dilation, only minimal data can be offered concerning the efficacy of the dilation of any or all of these lesions. The registry data do, however, demonstrate the safety of these dilations compared to alternative surgical therapy.

Complete transposition of the great arteries: treatment in the current era.

Transposition of the great arteries usually can be diagnosed by fetal echocardiography, which is important because the arterial switch operation, currently the treatment of choice, must be performed very early in life. The results of the arterial switch operation have been good to date. The dilemma remains, however, whether a fetus in whom a diagnosis of transposition is made should be aborted or treated surgically soon after delivery.

Fetal death after successful conversion of fetal supraventricular tachycardia with digoxin and verapamil.

A case of nonimmune hydrops fetalis associated with a supraventricular tachycardia was successfully treated with digoxin and verapamil. After resolution of the hydropic changes, however, the fetus unexplainedly died. Side effects from the drug therapy may have been responsible for the event.

Intraventricular tunnel repair for Taussig-Bing heart and related cardiac anomalies.

Uncertainty currently exists as to the optimal definitive procedure for treatment of patients with the Taussig-Bing heart and related cardiac anomalies. Between December 1, 1981 and October 1, 1985, we have used a completely intraventricular tunnel repair, without the use of an extracardiac conduit, in nine patients between 1 and 33 months of age. There was one hospital death from low cardiac output in a 1-month-old infant and one late death 11 months after repair from preexisting and progressive right ventricular cardiomyopathy. Six patients had the Taussig-Bing heart and three had transposition of the great arteries with a ventricular septal defect (VSD). The specific type of tunnel used was determined by the intracardiac anatomy and VSD enlargement was necessary in six patients. Three reoperations were required, two for recurrent VSD between 1 and 3 months after repair and one for tricuspid valve replacement in the patient with right ventricular cardiomyopathy 6 months later. The remaining seven patients are in NYHA class I 1.3 to 47 months postoperatively. We conclude that continued and perhaps expanded use of this technique is appropriate.

Spectrum of hearts with one underdeveloped and one dominant ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983. The age at first catheterization ranged from one day to 24 years (mean 3.9 years). Male-female ratio was 2:1. Five groups of hearts (with their relative frequencies in the spectrum) were established: dominant left ventricle (53%); dominant right ventricle (20%), each with normally related chambers; dominant left ventricle (20%); dominant right ventricle (3%), each with ventricular inversion; true single ventricle (5%). The incidence of atrial anatomy, venous return, intracardiac connections and associated lesions within each group was assessed. From the standpoint of deranged physiology as well as surgical implications there are more similarities than differences among these hearts. The fact that one ventricle will not generate an adequate stroke volume after repair is overwhelmingly more important than most other considerations. For the diagnostic and surgical approach, we believe that the system offers many advantages.

Repair of congenital pulmonary venous stenosis with living autologous atrial tissue.

Repair of localized bilateral congenital pulmonary venous stenosis with living autologous atrial tissue was accomplished in two patients, 3 and 4 months of age. The tip of the left atrial appendage was opened and used as an onlay patch to widen the stenotic area of the left pulmonary veins, leaving a double pathway for venous return. A flap of interatrial septum was evaginated through an incision in the right side of the left atrium and used as an onlay patch to widen the stenotic area of the right pulmonary veins. Effective relief of obstruction was demonstrated by nearly normal postrepair pulmonary artery pressure in each and early postoperative angiography in one. Although we believe this method of repair will increase the probability of a lasting result, further experience and subsequent late reevaluation are needed to demonstrate its long-term effectiveness.