Intracardiac undifferentiated sarcoma in infancy.

A rare case of an intracardiac undifferentiated sarcoma in a 3 month old infant is described together with the clinical, angiographic, echocardiographic, surgical and histopathologic findings. The tumor was successfully removed surgically, and monthly echocardiographic follow-up is being performed.

Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography.

Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of greater than or equal to 16 mm Hg at any level of the pulmonary venous return or of the systemic ventricular inflow. Severe obstruction was predicted if Doppler examination measured a flow velocity of greater than or equal to 2 m/s across any area of inflow obstruction. At catheterization, 12 patients (39%) had severe obstruction to left ventricular inflow or pulmonary venous return and all obstructions were correctly detected by Doppler echocardiography. The site of pulmonary venous obstruction was localized by two-dimensionally directed pulsed Doppler study. Patients with a lesser degree of obstruction had a lower Doppler velocity, but none had a maximal Doppler velocity of greater than or equal to 2 ms/s.(ABSTRACT TRUNCATED AT 250 WORDS)

Double balloon technique for dilation of valvular or vessel stenosis in congenital and acquired heart disease.

Despite the generally excellent success with balloon dilation for the stenotic lesions of congenital and acquired heart disease, technical difficulties sometimes prevent satisfactory results. Such technical difficulties include: a large diameter of the anulus of the stenotic lesion relative to available balloon diameter, difficulty in the insertion or removal of the larger balloon catheters, and permanent damage to or obstruction of the femoral vessels by the redundant deflated balloon material of the large balloons. A double balloon technique was initiated to resolve these difficulties. With this method, percutaneous balloon angioplasty catheters were inserted in right and left femoral vessels, placed side by side across the stenotic lesion and inflated simultaneously. Dilation procedures using the two balloon technique were performed in 41 patients: 18 with pulmonary valve stenosis, 14 with aortic valve stenosis, 5 with mitral valve stenosis, 3 with vena caval obstruction following the Mustard or Senning procedure and 1 with tricuspid valve stenosis. Patient ages ranged from 1 to 75 years (mean 17.8) and patient weights ranged from 8.9 to 89 kg (mean 42.3). Balloon catheter sizes ranged from 10 to 20 mm in diameter. Average maximal pressure gradient in mm Hg before dilation was 61 in pulmonary stenosis, 68 in aortic stenosis, 21 in mitral stenosis, 12 in tricuspid stenosis and 25 in vena caval stenosis. Average maximal valvular pressure gradient after dilation was 13 in pulmonary stenosis, 24 in aortic stenosis, 4 in mitral stenosis, 0 in tricuspid stenosis, and 1 in vena caval stenosis. No major complications were encountered with the procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

Results of balloon valvuloplasty in typical and dysplastic pulmonary valve stenosis: Doppler echocardiographic follow-up.

To assess the usefulness of balloon valvuloplasty in patients with a dysplastic pulmonary valve, the files of 36 patients (aged 1 day to 18.5 years) who had two-dimensional echocardiography before and continuous wave Doppler echocardiography late after balloon valvuloplasty (balloon diameter greater than or equal to 20% anulus diameter) were reviewed. Results of relief of pulmonary stenosis were graded by catheter gradient in the catheterization laboratory and compared with Doppler echocardiographic findings at follow-up. There were 32 patients with typical pulmonary stenosis and 4 with a dysplastic valve. In the 32 patients with typical pulmonary stenosis, transvalvular gradient changed from a mean of 67 +/- 32 to 20 +/- 20 mm Hg (p less than 0.0001, mean reduction 72.6%). The gradients at follow-up by Doppler echocardiography averaged 20 mm Hg including 15 that increased, 3 that were unchanged and 14 that decreased. Only 3 (9%) of 32 patients had a gradient greater than 25 mm Hg at follow-up and only one gradient was greater than 35 mm Hg. All four patients with a dysplastic valve had a gradient that decreased with valvuloplasty from a mean of 85 +/- 33 to 33 +/- 20 mm Hg (p less than 0.05); gradient reduction in this group ranged from 40 to 85% (mean 57.5%). The gradient at follow-up increased in three of these four patients and decreased in one (the only late gradient less than 25 mm Hg). Late gradient was less than 35 mm Hg in two of the four patients and was reduced by 43 and 57%, respectively, in the other two.(ABSTRACT TRUNCATED AT 250 WORDS)

Follow-up of coil occlusion of patent ductus arteriosus.

OBJECTIVE: We sought to determine the prevalence and fate of residual ductal shunting following coil occlusion of patent ductus arteriosus. BACKGROUND: Although transcatheter coil occlusion of patent ductus arteriosus has gained popularity, few follow-up data have been reported. METHODS: A review of 75 patients who underwent coil occlusion was performed. Residual shunting was investigated by Doppler echocardiography in follow-up. Angiograms were reviewed to obtain minimal ductal diameter and ductal angiographic type. RESULTS: Residual shunts were found in 31 patients (41%) on the day of the procedure, and of these, spontaneous closure was noted in 17 (55%) at 2 weeks to 20 months of follow-up. Of the 75 patients studied, 5 (7%) required a second coil procedure, and 10 (13%) remained with persistent residual shunts at most recent follow-up. Actuarial analysis estimated a 6 +/- 5% prevalence of residual shunts 20 months after a single coil procedure and 3 +/- 3% after all coil procedures. Minimal ductal diameter was associated with immediate complete ductal occlusion by a single coil. These patients had significantly smaller (p = 0.003) minimal ductal diameters (1.2 +/- 0.7 mm) than those who required two coils during their initial procedure to achieve immediate occlusion (1.9 +/- 0.7 mm), those who required a second coil procedure (2.0 +/- 0.9 mm), those who had spontaneous closure of residual shunts (1.9 +/- 0.7 mm) and those with persistent residual shunts (2.0 +/- 0.9 mm). No association was identified between ductal angiographic type and outcome of coil occlusion. No late adverse clinical events of coil occlusion or evidence of recanalization was found. CONCLUSIONS: Small residual shunts are common after coil embolization of patent ductus arteriosus, but most close spontaneously. Actuarial analysis estimates complete closure in 94% at 20 months, and reintervention was required in only 7% of patients.

Delineation of site, relative size and dynamic geometry of atrial septal defects by real-time three-dimensional echocardiography.

OBJECTIVES: This study attempted to determine the site, relative size and dynamic geometry of atrial septal defects using dynamic three-dimensional echocardiography. BACKGROUND: Recent studies have demonstrated the feasibility of dynamic three-dimensional echocardiography. Images are acquired from computerized reconstruction of sequential, tomographic ultrasound "slices" of the heart. Ultrasound images can be obtained by linear progression of a transducer within a transesophageal imaging probe. In small infants and children the large transducer size has not allowed transesophageal placement, and the probe has been placed on the thorax or in the subcostal position. Other scanning devices, housed in plastic containers, acquire images in a rotational format and can also be placed in a transthoracic or subcostal position. METHODS: Specially designed transesophageal probes and a dedicated computer unit were used for two-dimensional image retrieval and reconstruction of three-dimensional images. Sixteen patients with atrial septal defects were studied (median age 18 months, range 1 day to 18 years). In one patient, images were obtained by transesophageal probe placement; in the other 15 patients, the probe was placed in the transthoracic or subcostal position. RESULTS: A dynamic three-dimensional echocardiogram of the atrial septal defect could be obtained in 13 of the 16 patients. The distinguishing features of the atrial septal defects and their spatial orientation could be visualized in unique three-dimensional views. CONCLUSIONS: Dynamic three-dimensional imaging could be applied to the specific evaluation of atrial septal defects. Unique views of the heart allowed for spatial comprehension of the defects, rendering potentially important clinical information.

Continuous-wave Doppler in children with ventricular septal defect: noninvasive estimation of interventricular pressure gradient.

Continuous-wave Doppler was used to estimate the pressure gradient between the right and left ventricles in 28 children with ventricular septal defect (VSD). Doppler measurement of maximal velocity was performed during cardiac catheterization and the Doppler-predicted gradient was compared with the peak-to-peak gradient measured simultaneously by catheter. Doppler gradients ranged from 10 to 71 mm Hg and correlated well with measured gradient (r = 0.97, p greater than or equal to 0.001). Fourteen patients had isolated VSD, and in these patients Doppler measurements of gradient allowed accurate estimation of right ventricular pressure (r = 0.93). There was an inverse correlation between the ratio of pulmonary to systemic resistance and maximal velocity (r = -0.77). Thus, continuous-wave Doppler is an accurate means of measuring instantaneous VSD pressure gradient in children with congenital heart disease and can be used to estimate the right ventricular and pulmonary artery pressure in children with isolated VSD. This noninvasive method can be used to distinguish restrictive from nonrestrictive VSD.

Evaluation of a noninvasive index of global ventricular function to predict rejection after pediatric cardiac transplantation.

A Doppler myocardial performance index, defined as the sum of the isovolumetric contraction and relaxation time divided by the ejection time (ICT + IRT/ET), reflects global cardiac function, and when applied to the left ventricle, may serve as a predictor of moderate rejection in pediatric cardiac transplant patients.

Usefulness of color-flow Doppler in diagnosing and in differentiating supracristal ventricular septal defect from right ventricular outflow tract obstruction.

Color-flow Doppler is used for special localization of cardiac flows. The transeptal jet in supracristal ventricular septal defect (VSD) is directed toward the pulmonary valve and is often difficult to distinguish from the right ventricular (RV) outflow tract flow. Pulsed- and continuous-wave Doppler can misread the VSD jet as a stenotic lesion in the RV outflow tract. This study describes the color-flow Doppler characteristics of supracristal VSD and determines if color-flow Doppler can differentiate supracristal VSD jets from RV outflow tract flow. The study group comprised 28 patients ranging in age from 3 days to 23 years (mean 6.4), with catheter-diagnosed supracristal VSD in 14, isolated pulmonary valve stenosis in 10 and RV infundibular obstruction in 4. The echocardiographic Doppler and color-flow Doppler data of all patients were reviewed without knowledge of catheterization diagnosis. In all patients with supracristal VSD, color-flow Doppler revealed an abnormal transeptal jet directed toward the pulmonary valve that occurred 5 to 10 ms before RV outflow tract flow was identified. VSD and RV outflow tract jet could be differentiated by pulsed- and continuous-wave Doppler alone in 10 of 14 patients (71%). In 4 patients, differentiation was possible only with color-flow Doppler and electrocardiographic gating. Associated aortic regurgitation, or a fistula, or both, were detected in 6 patients. Pulsed- and continuous-wave Doppler with or without color-flow Doppler correctly identified the level of obstruction in all patients with RV outflow tract lesions (14 of 14). Supracristal VSD jet occurs in early systole and is directed toward the pulmonary valve.(ABSTRACT TRUNCATED AT 250 WORDS)

Fontan fenestration closure in the catheterization laboratory--echocardiographic evaluation of residual right to left shunts.

This study is a retrospective review using transthoracic echocardiography to assess the success of fenestration closure as well as residual right to left shunts in 35 patients who underwent Fontan fenestration closure in the catheterization laboratory. There is a high rate of closure of the Fontan fenestration; however, other residual right to left shunts are common.

Transcatheter occlusion of patent ductus arteriosus in adults.

This study examines whether transcatheter closure of a patent ductus arteriosus (PDA) using a Rashkind PDA occluder device is safe and effective in adults, or if adults have complications not sited in children owing to prolonged aorticopulmonary communication, high surgical risks or calcified PDAs. Fifteen patients aged 22 to 76 years (mean 42 +/- 14) were referred for transcatheter PDA occlusion. Exercise intolerance was the most frequent clinical manifestation. Eleven of 15 patients had surgical risk factors that included left ventricular failure (n = 10), biventricular failure (n = 1), elevated pulmonary pressures (n = 1), and a calcified PDA (n = 5). Twelve millimeter devices were placed in 4 PDAs < or = 3 mm in diameter; 17 mm devices were placed in 11 PDAs 3 to 6 mm in diameter. Seven (47%) were occluded angiographically shortly after device placement; another 5 PDAs (33%) were occluded echocardiographically within 24 hours of the procedure. Completed occlusion in this time interval was more likely to occur in PDAs < 5 mm in diameter (p = 0.0009). Of the 3 remaining PDAs with follow-up ranging from 9 to 38 months, 2 have demonstrated gradual diminution of shunting and have trivial leaks by color/Doppler flow. The other patient with a residual PDA has no ductal flow after placement of a second device. No complications related to device implantation or closure of the PDA occurred in any patient. No complications were reported in the follow-up patients who received evaluation (14 of 15 patients; range 1 to 38 months).(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary artery size and flow disturbances after patent ductus arteriosus coil occlusion.

Transcatheter closure of patent ductus arteriosus with Gianturco coils may impinge on adjacent vascular structures. The left pulmonary artery relative size may decrease after patent ductus arteriosus coil occlusion; thus, serial follow-up echocardiography is recommended to assess long-term left pulmonary artery growth.

Comparison of simultaneous Doppler- and catheter-derived right ventricular dP/dt in hypoplastic left heart syndrome.

Doppler estimation of RV dP/dt correlates well with micromanometer catheter-measured values in children with hypoplastic left heart syndrome. Doppler estimation of RV dP/dt is a method of quantifying RV systolic function independent of geometric assumptions, and may be a valuable method for longitudinal analysis of RV function.

Q-T prolongation and polymorphous ("torsade de pointes") ventricular arrhythmias associated with organophosphorus insecticide poisoning.

It is not generally appreciated in the Western world that organophosphorus poisoning may be associated with a serious and often fatal cardiac complication: Q-T interval prolongation with malignant ventricular arrhythmias of the "torsade de pointes" type. This insidious complication may lead to delayed, sudden death after the patients appears to be well on the way to recovery from the other, more dramatic respiratory and neurologic symptoms. In this study 15 patients with organophosphorus poisoning are described. Q-T prolongation was observed in 14 and malignant tachyarrhythmias in 6. In view of the dismal prognosis of these patients, ventricular pacing, previously used with success in other conditions associated with this syndrome, was tried in four patients and successfully shortened the Q-T interval and eliminated the arrhythmias. Isoproterenol did the same in a fifth patient. Awareness of this lethal, but preventable complication of organophosphorus poisoning is called for. Careful electrocardiographic monitoring is necessary until the Q-T interval returns to normal. Electrical pacing appears to be the treatment of choice for the tachyarrhythmias.

Improving results with first-stage palliation for hypoplastic left heart syndrome.

Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.

Critical aortic stenosis in the neonate. A comparison of balloon valvuloplasty and transventricular dilation.

The optimal treatment of critical aortic stenosis in the neonate and infant remains controversial. We compared transventricular dilation using normothermic cardiopulmonary bypass and percutaneous balloon aortic valvuloplasty with respect to early and late survival, relief of aortic stenosis, degree of aortic insufficiency, left ventricular function, and freedom from reintervention. Between July 1987 and July 1993, 30 neonates and infants underwent transventricular dilation or balloon aortic valvuloplasty for critical aortic stenosis. The patients in the transventricular dilation group (n = 21) ranged in age from 1 to 59 days (mean age 18.0 days +/- 19.1 days) and the balloon aortic valvuloplasty group (n = 9) from 1 to 31 days (mean age 10.0 days +/- 9.0 days). There were no significant differences in weight, body surface area, or aortic anulus diameter between the two groups (p = 1.0). Associated cardiovascular anomalies were more common in the transventricular dilation group (48%) than in the balloon aortic valvuloplasty group (11%). After intervention, the degree of residual aortic stenosis and insufficiency was equivalent in the two groups as assessed by postprocedural Doppler echocardiography. Ejection fraction improved within both groups (transventricular dilation 39% +/- 20.2% versus 47% +/- 22.0%; balloon aortic valvuloplasty 51% +/- 16.1% versus 62% +/- 8.4%), and there was no significant difference between groups. The left ventricular mass/volume ratio increased within both groups (p < 0.05) but with no significant difference between groups (transventricular dilation 1.4 +/- 0.5 gm/ml versus 1.8 +/- 0.6 gm/ml; balloon aortic valvuloplasty 1.1 +/- 0.6 gm/ml versus 1.7 +/- 0.4 gm/ml). Early mortality in the transventricular dilation group was 9.5% and in the balloon aortic valvuloplasty group, 11.1%. There was one late death in the transventricular dilation group. Four patients from the transventricular dilation group (19%) and two patients from the balloon aortic valvuloplasty group (22%) required reintervention for further relief of aortic stenosis. We conclude that both transventricular dilation and balloon aortic valvuloplasty provide adequate and equivalent relief of critical aortic stenosis. The treatment strategy adopted should depend on other factors, including associated cardiovascular anomalies, vascular access, preoperative condition, and the technical expertise available at each institution.

Idiopathic aldosteronism in pregnancy.

This is the first reported case of the idiopathic type of primary aldosteronism in pregnancy. The severely hypertensive patient was unresponsive to treatment with high doses of four antihypertensive agents administered concurrently. A drastic improvement in blood pressure was noted within 24 hours of beginning enalapril maleate, although subsequent deterioration in fetal status led to delivery at 26 weeks' gestation. Alternatives to standard medical therapy may be necessary for this rare but potentially life-threatening disease during pregnancy.

Modified Norwood operation for single left ventricle and ventriculoarterial discordance: an improved surgical technique.

BACKGROUND: Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block. METHODS: Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed. RESULTS: There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 +/- 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure. CONCLUSIONS: In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block.

May-Hegglin anomaly in pregnancy. A case report.

The May-Hegglin anomaly is a rare autosomal dominant platelet disorder that can be associated with significant bleeding. We treated a patient for pregnancy complicated by the May-Hegglin anomaly and a complete placenta previa.

Nonimmune hydrops fetalis and fetal congenital syphilis. A case report.

Nonimmune hydrops fetalis occurred secondary to a syphilitic infection. Ultrasonographic evaluation and cordocentesis were used to confirm the antenatal infection. An IgM antibody specific for Treponema pallidum wall antigen (anti-47-kDa) was used to document the fetal infection. High-dose intravenous penicillin was administered in an attempt to achieve adequate fetal levels.