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Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle.

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.

Cardiac Catheterization↗

Balloon pulmonary valvuloplasty in a child with Ebstein's anomaly and valvar pulmonary stenosis.

We performed a balloon pulmonary valvuloplasty in a child with Ebstein's anomaly using the standard technique. The procedure reportedly has not been performed in the face of this defect and, although difficult, was safely and successfully accomplished. In patients with Ebstein's anomaly and right ventricular outflow obstruction compounded by pulmonary valve stenosis, application of this technique may delay the need for surgical intervention.

Catheterization↗

Evaluation of a new finger-type intraoperative epicardial echocardiographic probe for infants and small children.

A new finger-type epicardial echocardiographic probe was evaluated in 26 children with complex cardiac anomalies during open heart surgery. The probe is a small 3.75 MHz phased array transducer for 2-dimensional echo and color Doppler imaging. The tip is slightly angled to facilitate the apical approach through a median sternotomy. The patients included 4 neonates and 8 infants, and the major cardiac lesions were complete atrioventricular (AV) canal, transposition of the great arteries, double outlet right ventricle, and others. The intraoperative study was possible in most of the patients facilitating various approaches for the assessment of left-sided AV valve function, left ventricular outflow obstruction, residual shunt and intraatrial anatomy. Four patients received subsequent operative procedures after the assessment. This transducer appears to be useful in the intraoperative study during surgery for complex cardiac anomalies in neonates and small infants.

Child↗

Late assessment after biventricular repair for isomerism heart.

BACKGROUND: Although biventricular repair is theoretically ideal for the treatment of isomerism heart, the long-term outcome is unknown. We assessed the outcome of biventricular repair for atrial isomerism. METHODS: From 1984 to 2002 in our surgical database, 10 of 67 patients with atrial isomerism received biventricular repair. The age at operation was 7.7 +/- 9.1 years. Preoperative ventricular volume was normal in all patients. Left ventricular ejection fraction was 62% +/- 8%. Intra-atrial rerouting was required in 8. The atrioventricular septal defect with double-outlet right ventricle was closed using comma-shaped intraventricular conduit in 8. Other procedures included pulmonary valvotomy and infundibulectomy in 2, transannular patch in 1 and right ventricle to pulmonary artery conduit in 1. RESULTS: There was one early death due to hemolytic phagocytic syndrome. The other 9 patients are in New York Heart Association class I at 12.7 +/- 5.3 years postoperatively. There was no atrial baffle stenosis except in 1 patient; it was successfully treated by stent placement 10 years after the operation. There was no incidence of left ventricular outflow obstruction. Mitral replacement (4 months postoperatively) or repair (15 years postoperatively) was done in 2 patients. The other 5 patients with atrioventricular septal defect showed trivial to mild regurgitation in the long-term period. Arrhythmia was observed in 5 (left 4, right 1). Catheter ablation was needed in a patient with atrial flutter. Three of 9 patients require diuretics or digitalization, or both. CONCLUSIONS: The long-term outcome of the biventricular repair for atrial isomerism was excellent. Late development of mitral regurgitation and arrhythmia could be managed adequately.

Adolescent↗

Hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is a fascinating disease of marked heterogeneity. Hypertrophic cardiomyopathy was originally characterized by massive myocardial hypertrophy in the absence of known cause, a dynamic left ventricular outflow obstruction, and increased risk of sudden death. It is now well accepted that multiple mutations in genes encoding for the cardiac sarcomere are responsible for the disease. Complex morphologic and pathophysiological differences, disparate natural history studies, and novel treatment strategies underscore the challenge to the practicing cardiologist when faced with the management of the patient with hypertrophic cardiomyopathy.

Cardiomyopathy, Hypertrophic↗

Traumatic intramyocardial left ventricular dissection: a case report.

In this report, we present an unusual case of a patient who sustained and survived an intramyocardial dissecting hematoma with subacute ventricular rupture complicating a motor vehicle accident. In conclusion, we report on the diagnosis, management, and prior literature of this highly unusual and often lethal condition.

Accidents, Traffic↗

Arterial switch operation with in situ coronary reallocation for transposition of great arteries with single coronary artery.

OBJECTIVES: Transposition of great arteries (TGA) with single coronary artery pattern is one of the high-risk groups for arterial switch operation (ASO). Any traction or kinking during coronary transfer can lead to a fatal outcome. With increase in experience, surgical results improved, but it did not completely eliminate the risks of coronary translocation. Many techniques have been described for transfer of single coronary and each one has its own merits and problems. We here in describe a new technique of in situ coronary reallocation during ASO for TGA with single coronary and also report the early and mid-term results with this new technique. METHODS: From September 1988 to June 2002, five consecutive cases of TGA with single coronary artery were operated employing this new technique. Their age ranged from 16 days to 9 months. ASO was done by transecting the great arteries just above the commissures. For coronary reallocation, hockey stick-shaped incisions were made in the facing sinuses of the proximal aorta and the pulmonary artery. These flaps were sutured in such a way that the coronary ostium was committed to the neo-aorta with the rest of surgical procedure done in the usual manner. RESULTS: All five patients had ASO. Additionally, four patients had closure of an associated ventricular septal defect and one patient had repair of the coarctation of the aorta. There was no in hospital mortality. All patients had follow-up echocardiograms at regular intervals, which showed no significant right or left ventricular outflow obstruction, no regional wall motion abnormalities and no neo-aortic or neo-pulmonary regurgitation. Three of five patients had cardiac catheterization and angiocardiography, which showed normal coronary arteries with no obstructive lesions and no neo-aortic regurgitation. Their follow up ranged from 5 to 50 months and there was no late mortality. CONCLUSIONS: This new coronary reallocation technique avoids problems related to coronary translocation such as traction and kinking. It spares the need for dissection of proximal coronary artery and its branches, and thereby eliminates the risk of development of fibrosis and stenosis. The same technique can be used regardless of the sinus of origin of the coronary artery. It is a reliable and a reproducible technique. The early and mid-term results appear excellent in this series.

Aortography↗

Outcomes of pregnancy in women with tetralogy of Fallot.

OBJECTIVES: We sought to determine pregnancy outcomes in patients with tetralogy of Fallot (TOF). BACKGROUND: Pregnancy outcomes in patients with TOF are incompletely defined. METHODS: Clinical, hemodynamic, and obstetric data were reviewed for women with TOF and prior pregnancy. RESULTS: Of 72 respondents, 43 (mean age, 26 years) had 112 pregnancies (range, 1 to 5); 82 pregnancies were successful. Eight women had unrepaired TOF at the time of their 20 successful pregnancies. At first assessment (age > or =18 years), six patients had pulmonary hypertension, three had moderate or severe right ventricular (RV) systolic dysfunction, and 13 had severe RV dilation due to pulmonic regurgitation. Sixteen patients had 30 miscarriages (27%) and one term stillbirth. Mean overall birth weight was 3.2 kg (range, 2.1 to 4.2 kg). Unrepaired TOF (p = 0.05) and morphologic pulmonary artery abnormality (p = 0.03) were independently predictive of infant birth weight. Six patients had cardiovascular complications during pregnancy: supraventricular tachycardia in two, heart failure in two, pulmonary embolism in a patient with pulmonary hypertension, and progressive RV dilation in a patient with severe pulmonic regurgitation. Five infants (6%) had congenital anomalies. CONCLUSIONS: Patients with TOF have an increased risk of fetal loss, and their offspring are more likely to have congenital anomalies than offspring in the general population. Adverse maternal events, although rare, may be associated with left ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with RV dysfunction.

Adult↗

Electrocardiogram in double chamber right ventricle.

Thirty patients with a double chamber right ventricle were seen during a 7 year period. The majority of patients had associated cardiac anomalies, most commonly a ventricular septal defect. In this series, as in others, the history, clinical examination, chest roentgenogram and echocardiogram were inconclusive for diagnosing double chamber right ventricle. Symptoms were generally related to the severity of the associated anomalies. In 40 percent of the patients reviewed, upright T waves were found in lead V3R as the only electrocardiographic finding suggestive of right ventricular hypertrophy. Because upright T waves in lead V3R in the absence of other evidence of right ventricular hypertrophy are not commonly seen in patients with an isolated ventricular septal defect or a defect associated with other forms of right ventricular outflow obstruction, this finding should alert the cardiologist to the possibility of the presence of double chamber right ventricle prior to cardiac catheterization.

Adolescent↗

Tetralogy of Fallot and coarctation of the aorta: successful repair in an infant.

A 4 1/2-month-old infant was referred to our center with the rare combination of tetralogy of Fallot and coarctation of the aorta. Despite the presence of severe right ventricular outflow obstruction, the infant was virtually acyanotic because of the systemic afterload associated with the coarctation. She had marked systemic hypertension. The embryological and therapeutic implications of this child's defects are discussed.

Aortic Coarctation↗

Hemodynamic effects of various modes of cardiac pacing after operation for idiopathic hypertrophic subaortic stenosis.

The hemodynamic effects of varying heart rate and pacing site were studied in 6 patients with idiopathic hypertrophic subaortic stenosis following operative relief of outflow obstruction. Ventricular pacing (117 beats per minute) resulted in a 26% decrease in cardiac output (p less than 0.02), a 54% increase in pulmonary capillary wedge pressure (p less than 0.03), and a 23% decrease in mean blood pressure (p less than 0.05), compared with normal sinus rhythm (88 beats per minute). Slow atrial pacing (112 beats per minute) did not significantly alter any hemodynamic variable compared with normal sinus rhythm. Rapid atrial pacing (143 beats per minute) produced a similar degree of hemodynamic impairment as ventricular pacing. This study demonstrates that ventricular pacing at heart rates commonly used clinically and rapid atrial rates result in a significant fall in cardiac output. Preservation of atrial systole at heart rates that allow adequate diastolic ventricular filling of a hypertrophied, noncompliant ventricle is stressed. In addition, atrial electrodes are useful to record atrial electrograms or induce rapid atrial stimulation to treat supraventricular tachyarrhythmias.

Adult↗

Interrupted aortic arch in infancy.

Data from 30 infants with interrupted aortic arch in the New England Regional Infant Cardiac Program, 1968 to 1974, were reviewed. All patients had major associated cardiac abnormalities: approximately one third had ventricular septal defect with patent ductus arteriosus, one third had complex ventricular septal defect with left ventricular outflow obstruction, and one third had complex intracardiac lesions incompatible with survival. Twenty-one of the patients had either palliative or reconstructive surgery, with a hospital mortality rate of 76%. Nine patients did not have surgery; eight of them died at a median age of four days. One-stage primary repair of interrupted aortic arch including the associated cardiac defects, using deep hypothermia and circulatory arrest, is proposed as the current method of treatment in such infants.

Aorta↗

Administration of prostaglandin E1 in neonates with critical congenital cardiac defects.

Prostaglandin E1 was administered to 12 neonates and young infants in whom pulmonary or systemic blood flow was entirely or significantly dependent upon persistent patency of the ductus arteriosus. Nine neonates responded favorably to PGE1 infusion; their mean age was 2.8 days. Three infants who failed to respond were 10 days, 14 days, and 9 weeks of age, respectively. Six neonates with right ventricular outflow obstruction had a mean increase in arterial PO2 of 136% following administration of PGE1. In three patients in whom systemic flow was ductus dependent, PGE1 infusion was followed by improvement in arterial blood pressure, peripheral perfusion, and urine output. Complications included pyrexia, vasodilatation, and myoclonic jerks (or focal seizures). Three side effects were easily reversible by decreasing the infusion rate or altering the site of administration. PGE1 is a highly effective agent in stabilizing critically ill neonates with ductus dependent congenital cardiac lesions.

Blood Pressure↗

Noninvasive assessment of surgical systemic to pulmonary artery shunts by range-gated pulsed Doppler echocardiography.

Suprasternal range-gated pulsed Doppler echocardiography was used to evaluate surgical shunt patency in children with cyanotic congenital heart disease and right ventricular outflow obstruction following systemic artery to pulmonary artery anastomoses. Normal patients had laminar Doppler auditory signals and time interval histogram flow patterns because right pulmonary artery turbulence was not present during systole or diastole. Patients with pulmonary valvar stenosis had turbulence during systole only. In all nine postoperative patients with surgical shunts, turbulent systolic and diastolic time interval histograms and abnormal auditory signals were present. A typical shunt murmur was not audible to auscultation in six of the postsurgical patients at the time of the study. Suprasternal range-gated pulsed Doppler echocardiography affords an accurate, safe and simple noninvasive bedside means for confirming shunt patency in patients who have undergone surgical systemic artery to pulmonary artery anastomoses.

Adolescent↗

Use of bovine jugular vein to reconstruct the right ventricular outflow tract: early results.

OBJECTIVE: We evaluate early results of bovine jugular vein conduits in the pulmonary outflow. METHODS: Between April 2000 and September 2001, 31 conduits were placed in the outflow of the right ventricle. Patients who received a conduit as a staged surgical procedure were excluded (n = 3). Implantation age ranged from 0 to 21 years (median, 3.4 years). Conduit diameter ranged from 12 to 20 mm (median, 14 mm). Transthoracic echocardiography was performed at discharge and 3 months after surgery. Patients with significant pulmonary regurgitation and/or stenosis underwent cardiac catheterization. RESULTS: Four patients died during the follow-up period. Three deaths were unrelated to the conduit. One death was related to the complete thrombosis of the conduit. At 3 months evaluation, pulmonary valve regurgitation was absent or trivial in 19, mild in 2 and severe in 3 of 24 survivors. Four patients had nonfatal conduit-related complications. A transient thrombus formation within 1 leaflet was noted postoperatively in a patient with a moderate pulmonary regurgitation. Three patients required reoperation 3 to 5.8 months after the implantation for conduit failure (mean, 4.3 months). Cardiac catheterization before replacement revealed an aneurysmal dilation of the conduit below a severe stenosis of the pulmonary bifurcation due to important neointimal proliferation. CONCLUSIONS: Early failure of bovine jugular vein valved conduits can occur because of exaggerated intimal proliferation or thrombotic process within the conduit. Because of these complications, close echocardiographic follow-up is mandatory during the first weeks after implantation.

Adolescent↗