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Objectives: This study sought to determine risks and outcome of pregnancy and delivery after the modified Fontan operation., Background: Increasingly, female Fontan patients reaching child-bearing years are interested in having children. To date, the number of reported pregnancies is small, and pregnancy has therefore been discouraged., Methods: One hundred ten of 126 female patients from the Fontan registries of the Mayo Clinic and University of California Los Angeles Medical Center responded to a mailed questionnaire. An additional six patients with a reported pregnancy from other centers were identified and reviewed to assess pregnancy outcomes., Results: Among the participating centers, a total of 33 pregnancies after Fontan operation for various types of univentricular heart disease were reported. There were 15 (45%) live births from 14 mothers, with 13 spontaneous abortions and 5 elective terminations. In the 14 women with live births, the median number of years between operation and pregnancy was 4 (range 2 to 14). Reported prepregnancy problems in these gravidas included atrial flutter in one patient and ventricular dysfunction, aortic regurgitation and atrioventricular valve regurgitation in another. One patient developed supraventricular tachycardia during pregnancy and had conversion to sinus rhythm. No maternal cardiac complications were reported during labor, delivery or the immediate puerperium. There were six female and nine male infants (mean gestational age 36.5 weeks; median weight 2,344 g). One infant had an atrial septal defect. At follow-up, mothers and infants were alive and well., Conclusions: Pregnancy after the Fontan operation appears to have been well tolerated in 13 to 14 gravidas. There does appear to be an increased risk of miscarriage. The tendency to routinely discourage pregnancy may need to be reconsidered.

Objectives: The reliability of two-dimensional echocardiography for determining the proximal coronary artery anatomy in d-loop transposition of the great arteries was investigated in 406 infants who underwent surgical repair at one institution., Background: The origin and proximal course of the main coronary arteries can affect the surgical results of the arterial switch operation. Preoperative determination of the coronary artery anatomy appears to be advantageous for the surgeon., Methods: All infants with d-loop transposition who underwent a two-dimensional echocardiogram and primary surgical repair at our institution between 1987 and 1992 were identified, and the echocardiographic, operative and, when available, autopsy reports were reviewed for coronary artery anatomy, presence of a ventricular septal defect and the spatial relation between the arterial roots. The two-dimensional echocardiographic findings were compared with surgical or autopsy findings. The relation between proximal coronary artery anatomy and 1) a ventricular septal defect, and 2) the spatial orientation of the arterial roots was investigated. Twenty-seven infants diagnosed with an intramural coronary artery were not included because they are the subjects of another report., Results: Excluding intramural coronary artery patterns, 10 different types of coronary artery anatomy were seen in these 406 patients. The coronary arteries were imaged adequately in 387 (95%) of the 406 patients. The coronary artery anatomy was determined correctly by two-dimensional echocardiography in 369 (95.4%) of the 387 patients, with 18 errors in diagnosis. During the most recent 2.5 years, 193 (98.5%) of 196 patients were diagnosed correctly, with three diagnostic errors. Patients with a ventricular septal defect or side-by-side great arteries are more likely to have an unusual coronary pattern., Conclusions: Echocardiography appears to be highly reliable for determining proximal coronary artery anatomy in d-loop transposition of the great arteries. An unusual coronary artery pattern is more likely in patients with side-by-side great arteries or posterior aorta or a ventricular septal defect, or both.

Objectives: This report describes transesophageal echocardiographic guidance of transcatheter closure of ventricular septal defects and its value as an adjunct to fluoroscopy and angiography in this procedure., Background: Experience with transcatheter closure of ventricular septal defects has identified a diverse group of patients in whom it may be the procedure of choice. Although facilitating other interventional procedures, such as transcatheter closure of atrial septal defects, the value of transesophageal echocardiographic guidance for transcatheter ventricular septal defect closure has not been documented., Methods: All patients who underwent ventricular septal defect closure with transesophageal echocardiographic guidance before November 1992 were included. Angiograms and echocardiograms were reviewed to evaluate device position and relation to valve tissue during placement and to assess residual flow after device implantation. The ability of transesophageal echocardiography to assess these variables was compared with fluoroscopy and angiography., Results: Transesophageal echocardiographic guidance was used in 31 of the 83 catheterizations involving transcatheter ventricular septal defect closure performed between February 1990 and November 1992. Under transesophageal echocardiographic guidance, 45 devices were implanted: 23 in muscular ventricular septal defects, 17 in residual postoperative patch margin defects and 5 in other ventricular septal defects. Transesophageal echocardiographic guidance enhanced assessment of device position and proximity to valve structures and markedly improved assessment of residual flow. Assessment of residual flow with transesophageal echocardiography eliminated the need for multiple angiograms in some patients. Combining transesophageal echocardiography with fluoroscopy and angiography provided the most information., Conclusions: Transesophageal echocardiography facilitates transcatheter closure of ventricular septal defects by improving assessment of device position and effectiveness of closure. It is indicated when device placement is likely to be difficult or may interfere with valve structures or when multiple interventional procedures are anticipated.

Background: Anomalous origin of the left coronary artery from the pulmonary trunk is difficult to diagnose reliably by two-dimensional echocardiography. Therefore, Doppler color flow mapping was tested in 29 patients with dilated cardiomyopathy or anomalous left coronary artery, or both., Methods and Results: All patients with anomalous left coronary artery (10 patients) or dilated cardiomyopathy (27 patients) (excluding those with other known causes for cardiomyopathy) examined between January 1988 and May 1991 were identified. The direction of flow in the three main segments of the left coronary system was determined by Doppler color flow mapping. In all 10 patients with anomalous left coronary artery, flow mapping demonstrated an abnormal jet from the left coronary artery into the pulmonary trunk and retrograde flow in at least two segments of the left coronary system. The diagnosis was confirmed in all 10 patients at operation. Doppler color flow mapping, performed in 19 of the 27 patients with dilated cardiomyopathy, demonstrated anterograde flow in at least one segment of the left coronary system in 16 of the 19 patients; flow direction was not determined in the other 3 patients. Coronary artery anatomy was confirmed by aortic root or left ventricular angiography in 14 patients and at autopsy in 1 patient and was not directly confirmed in 4 patients. Left ventricular function spontaneously improved to normal in three of the latter four patients, a clinical course not consistent with anomalous left coronary artery. The left coronary artery appeared to arise from the aortic root by two-dimensional echocardiographic imaging alone in all patients with dilated cardiomyopathy and in 5 of 10 patients with anomalous left coronary artery (50% false negative diagnoses)., Conclusions: Detection of an abnormal jet into the pulmonary trunk and retrograde flow in the left coronary system by Doppler color flow mapping is reliable for diagnosing anomalous left coronary artery whereas two-dimensional echocardiographic imaging alone is often inconclusive or misleading. Determining flow direction in the left coronary system in patients with dilated cardiomyopathy is useful for excluding anomalous left coronary artery but is technically more difficult to document in this condition than in anomalous left coronary artery.

Bulboventricular foramen obstruction may complicate the management of patients with single left ventricle. Bulboventricular foramen size was measured in 28 neonates and infants greater than 5 months old and followed up for 2 to 5 years in those patients whose only systemic outflow was through the foramen. The bulboventricular foramen was measured in two planes by two-dimensional echocardiography, its area calculated and indexed to body surface area. One patient died before surgical treatment. The mean initial bulboventricular foramen area index was 0.94 cm2/m2 in 12 patients (Group A) in whom the foramen was bypassed as the first procedure in early infancy. The remaining 15 patients underwent other palliative operations but the bulboventricular foramen continued to serve as the systemic outflow tract. There was one surgical death. Six (Group B) of the 14 survivors developed bulboventricular foramen obstruction during follow-up (mean initial bulboventricular foramen area index 1.75 cm2/m2). The remaining eight patients (Group C) did not develop obstruction during follow-up and had an initial bulboventricular foramen larger than that in the other two groups (mean initial bulboventricular foramen area index 3.95 cm2/m2). All patients with an initial bulboventricular foramen area index less than 2 cm2/m2 who did not undergo early bulboventricular foramen bypass developed late obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

The records of 23 infants who underwent surgical repair of isolated totally anomalous pulmonary venous connection were reviewed to assess the accuracy of pre- and postoperative echocardiographic diagnoses. Preoperative echocardiographic diagnoses were accurate in 22 of 23 patients, including the sites of connection of the individual pulmonary veins. Cardiac catheterization in 13 patients confirmed the echocardiographic findings. Analysis of multiple pre- and postoperative variables revealed no statistically significant difference between the infants with and without catheterization, although there was a tendency toward a higher mortality rate in the catheterized group. Postoperative echocardiographic examination revealed obstruction to pulmonary venous return in 7 of 19 patients. Catheterization confirmed the echocardiographic findings, localizing the obstruction in one patient. The size of the venoatrial anastomosis was measured on postoperative echocardiograms performed on 14 patients. The cross-sectional area of the anastomosis was less than 0.3 cm2/m2 of body surface area in the four patients with obstruction of the anastomosis, and greater than 0.95 cm2/m2 in all long-term survivors examined. Two-dimensional echocardiography with pulsed Doppler examination and Doppler color flow mapping is an excellent means of diagnosing totally anomalous pulmonary venous connection. The connections of the individual pulmonary veins can be identified in nearly all cases. Surgical repair can usually be undertaken on the basis of echocardiographic diagnosis alone. Echocardiography also provides an extremely accurate method of evaluating surgical repair and of identifying and localizing postoperative obstruction to pulmonary venous return.