Reopening after successful coil occlusion for patent ductus arteriosus.

OBJECTIVES: This study was performed to determine the frequency of patent ductus arteriosus (PDA) reopening and the factors that may predict reopening after successful coil occlusion. BACKGROUND: Transcatheter coil occlusion is a widely used and accepted method to close a PDA. After documented successful coil occlusion, we found PDAs that reopened. We hypothesized that specific factors are involved in those that reopened. METHODS: All patients who underwent percutaneous transarterial PDA coil occlusion were studied. Successful coil occlusion was documented. PDA reopening was determined when Doppler-echocardiography (DE) performed after the procedure was negative for PDA flow but at follow-up demonstrated PDA shunting. Patients with a reopened PDA were compared with all other patients in evaluating independent variables. RESULTS: Coil occlusion for PDA was attempted in 22 patients. Clinical success was achieved in 20 patients (91%), and DE was negative for PDA shunting in 19 patients (90%). At follow-up, five patients demonstrated reopening. The PDA minimal diameter was 1.4 +/- 0.5 mm (mean +/- SD) for the reopened group and 1.2 +/- 0.7 mm for the other patients. The PDA length was 2.9 +/- 1.9 mm for the reopened group and 7.1 +/- 3.2 mm for all other patients. All those with type B PDA were in the reopened group. When independent variables were compared between groups, only PDA length and type B PDA predicted reopening (p < 0.05). CONCLUSIONS: PDA reopening may occur after successful coil occlusion. Short PDA length and type B PDA are associated with reopening. The data suggest that in such anatomy, alternative strategies to the current coil occlusion technique should be considered.

Dicephalus dipus dibrachius: an unusual case of conjoined twins.

Conjoined twinning is a rare anomaly, occurring in one of every 50,000 births. Dicephalus dipus dibrachius is an extremely rare form of conjoined twinning in which the infant has two arms, two legs, one trunk, but two heads. These infants are often stillborn or die shortly after birth. The authors describe a case of dicephalus twinning with 11-day survival. A thorough investigation using multiple imaging modalities (plain radiographs, contrast studies, ultrasonography, and magnetic resonance imaging) demonstrated that these twins had separate spinal columns but shared multiple internal organs including heart, liver, pancreas, intestine and bladder. Based on the anatomy of this case, the authors conclude that separation of dicephalus dipus dibrachius twins should not be attempted.

Anomalous course of the left main coronary artery in tetralogy of Fallot.

An extremely rare coronary artery anomaly where the left main coronary artery arose anteriorly from the right coronary sinus and coursed in front of the right ventricular outflow tract was present in a patient with tetralogy of Fallot. Preoperative angiocardiography was interpreted as normal. Operative recognition was prevented by dense adhesions and a partial intramural course. Division of the vessel at repair resulted in death of the patient. The angiographic pattern associated with this anomaly is very unusual, and in many views looks deceptively normal. Details are presented.

Coronary artery fistula in the pediatric age group: a 19-year institutional experience.

Ten patients with coronary artery fistulae were identified from records at Columbus Children's Hospital between 1974 and 1993. Clinical presentations of patients were quite variable, from 1 day to 20 years of age. Symptoms ranged from none to severe cardiorespiratory failure requiring extracorporeal membrane oxygenation. Long term follow-up revealed one sudden death and one spontaneous closure of the fistula. This lesion should be ruled out in patients who present as extracorporeal membrane oxygenation candidates. Patients with mild forms of this lesion may be followed up medically if the left to right shunt is inconsequential, because spontaneous closure is a possibility. Because of the risk of sudden death, close long-term follow-up is mandatory even for operated patients, and antiplatelet therapy should be considered for these patients.

Cavopulmonary anastomotic aneurysm: a complication in pulsatile pulmonary arteries.

An aneurysm of a left superior vena cava developed after anastomosis to the left pulmonary artery during repair of an atrioventricular septal defect with a persistent left superior vena cava entering directly into the left atrium. We believe this implies caution should be used in accepting the recent suggestion of using a bidirectional cavopulmonary connection to a pulsatile pulmonary arterial circuit as a way of allowing anatomic correction of atrioventricular septal defect with a small right ventricle.

Use of cardiac catheterization in pediatric cardiac surgical decisions.

Current practice patterns relating to pediatric cardiac catheterization (Cath) have considerable economic implications. The decreased cost and risk of noninvasive methods such as echocardiography (ECHO) and magnetic resonance imaging (MRI) make them attractive alternative diagnostic methods if they can sufficiently define cardiac anatomy and the need for surgical intervention. We reviewed a recent cardiac surgical series of 465 cases in 1.5 years to determine how often a Cath was performed prior to surgery. Overall, 59.4% of the procedures were preceded by a Cath (76% of open heart operations, and 26.7% of closed heart operations). We specify the situations where we feel enough information is available for preoperative decision making from non-invasive testing, and we present some diagnostic pitfalls that have been encountered.

Alloantigen-dependent endothelial phenotype and lymphokine mRNA expression in rejecting murine cardiac allografts.

Recent studies suggest that graft microvascular endothelia may play an important role in the regulation of rejection. Alloantigen-dependent changes in microvascular endothelial phenotype may be associated with differences in infiltrate function in allografts vs. isografts, as reflected in alloantigen-specific CTL accumulation and cytokine production. To correlate cytokine production with differences in microvascular endothelial phenotype during allograft inflammation, we used PCR to identify cytokine mRNAs isolated from pooled cardiac isografts and allografts on days 1, 3, and 5 after transplantation. Graft microvascular endothelia express an inflamed phenotype associated with wound healing and the repair of tissue damage due to mechanical trauma, ischemia, and/or reperfusion injury--i.e., high levels of ICAM-1 expression and MECA-32 mAb reactivity. By day 1 in both isografts and allografts, mRNAs for the cytokines IL1 alpha, IL6, TNF, LT, and TGF beta are upregulated or induced. By the third day in cardiac allografts, an antigen-dependent endothelial phenotype is expressed, characterized by the presence of cell surface VCAM-1. Concomitantly, mRNAs for the lymphokines IL2 and IFN gamma are detected, followed by IL4 mRNA by day 5. The expression of VCAM-1 by allograft endothelia may influence the inflammatory process, by physically recruiting specific T cell subpopulations into the response and/or by delivering additional signals to the infiltrating cells. Eventually, these and other regulatory events occurring at these early times initiate a process that later results in alloreactive tissue destruction.

Palliative arterial repair for transposition, ventricular septal defect, and pulmonary vascular disease.

Failure to repair transposition of the great arteries and ventricular septal defect in the young infant results in the early development of pulmonary vascular occlusive disease. Complete repair, preferably by an arterial switch procedure and ventricular septal defect closure, may then not be possible. We report a palliative arterial switch procedure in a 5 1/2-year-old patient with transposition, ventricular septal defect, and severe pulmonary vascular obstructive disease in whom progressive hypoxemia and exercise intolerance developed. An arterial repair without ventricular septal defect closure was performed. After the operation, the child's systemic arterial oxygen saturation and exercise tolerance have substantially improved. Although the progression of pulmonary vascular disease may not be altered, arterial repair can provide effective palliation in this subset of patients.

Results of operation for aortic valve stenosis in infants, children, and adolescents.

Surgical procedures for aortic valve stenosis may be considered either corrective or palliative. During a 22-year period from 1962 to 1984, 120 patients required operation for aortic valve stenosis. The operations done included 117 valvotomies and three initial valve replacements. Six patients, five infants and a 7-year-old girl, died at operation. The remaining 114 patients were followed up for 1 to 23 years (mean 8.7 years). Twenty-six patients (23%) required a second operation 1 to 15 years (mean 6.8 years) after initial valvotomy. Six patients (5%) required a third operation 3 months to 8 years (mean 4.4 years) after the second operation. Eighteen of the 26 patients (69%) having second operations required valve replacement. All third operations were valve replacements. No perioperative deaths occurred at the second and third operations. There were four sudden late deaths (3.5%). Eighty-four of the 114 patients (74%) followed up for 1 to 23 years (mean 7.7 years) have had a satisfactory result from initial valvotomy, being free of symptoms and major events (stroke, endocarditis, sudden death), and have not required reoperation. Fifty-nine percent of a subgroup of 22 patients followed up for a mean of 17.7 years have had a satisfactory result from initial valvotomy.

Complete absence of pulmonary veins.

Various types of pulmonary venous return abnormalities have been described in the literature. This report presents a case in which a 4-h-old neonate presented with cyanotic heart disease and respiratory distress. This neonate was subsequently shown to have complete absence of the pulmonary veins (CAPV), a previously undescribed malformation. The case summary describes the physical findings, radiographic and electrocardiographic features, cardiac catheterization data, and results from autopsy. A discussion of the case, theories of embryological etiology for this malformation, and differential diagnoses follow. Surgical treatment for CAPV is not yet possible.

Resurgence of acute rheumatic fever.

Rheumatic fever has been disappearing in the United States, especially during the past two decades. In the past two years, however, there have been 40 patients seen at the Columbus (Ohio) Children's Hospital diagnosed as having acute rheumatic fever. In marked contrast to the infrequency during the preceding ten years. Twenty of the 40 patients had carditis. Five of these patients were suffering from heart failure, and there was one death. The cause for this outbreak has not yet been found, but some possible causes are discussed. A possible nationwide resurgence of this disease may be heralded by our experience and that of others.

Pedunculated left ventricular rhabdomyoma.

A patient with an unusual left ventricular outflow tract obstruction caused by a solitary pedunculated left ventricular rhabdomyoma is described. Diagnosis was based on two-dimensional echocardiographic findings alone. The obstructive portion of the tumor was successfully removed from the interventricular septum by an aortic root approach.

Air gun pellet injury of the heart with popliteal embolus.

An air gun pellet cardiac injury, in which there was penetration through the right ventricle, interventricular septum, and anterior papillary muscle and ejection from the left ventricle, is described. The pellet embolus was removed from the left popliteal artery with restoration of flow. The particular implications of pellet embolization are discussed and contrasted with those of bullet embolism.

Pulmonary valvulotomy in Factor XI deficiency.

This is the first reported case in the English literature of Factor XI deficiency in a child from India. He underwent open-heart surgery and pulmonary valvulotomy without excessive or delayed hemorrhage using plasma infusion to sustain Factor XI levels at about 40% of normal before, and for 10 days after surgery. The management of this child is presented as an approach to patients with Factor XI deficiency who require major surgery.

Supravalvular aortic stenosis. A 20-year clinical perspective and experience with patch aortoplasty.

Supravalvular aortic stenosis has a wide range of clinical and morphologic expression. Since 1961, 25 patients (aged 1 to 49 years) with documented supravalvular aortic stenosis have been evaluated. Seven (28%) had Williams' syndrome, 5 (20%) had a familial form of supravalvular aortic stenosis, and 13 (52%) had a sporadic form. A blood pressure difference of greater than 10 mm Hg between the arms was noted in 65% of the patients. Angiographically, 19 (76%) had segmental supravalvular narrowing; 6 (24%) had diffuse narrowing of the ascending aorta. Sixteen patients underwent patch aortoplasty. At surgery, portions of the aortic valve cusps were frequently attached to supravalvular tissue. This "cusp tuck" resulted in distinctive angiographic features and influenced the results of corrective surgery. Three surgical deaths occurred in the early 1960s-2 with diffuse narrowing of the aorta. Of the remaining 12 patients, followed for 1 to 12 years, 10 are asymptomatic, 1 has angina, and 1 died from cancer. All 8 patients who underwent postoperative catheterization had a thick band between the left and right coronary sinus which represented persistent attachment of portions of the aortic valve cusps to residual supravalvular tissue (cusp tuck). This resulted in aortic valvular gradients (23 to 48 mm Hg) in 4 patients and aortic valvular insufficiency in 2 patients. No significant supravalvular gradient was noted. The 20-year experience with supravalvular aortic stenosis reported herein emphasizes a wide range of clinical and morphologic expression, the benefits and limitations of patch aortoplasty, and the importance of postoperative cardiac catheterization, and furthers the understanding of a complex clinical syndrome.

Right atrial myxoma in a child.

A 12-year-old girl with fatigability, dyspnea, variable murmurs, and chronic congestive failure was treated for chronic myocarditis with medical management for 16 months. Cardiac catheterization and angiography performed to rule out constrictive pericarditis disclosed a right atrial myxoma. Following removal of the mass, this patient became asymptomatic. Since right atrial myxoma rarely occurs in children and can mimic other diseases, the diagnosis depends on a high index of suspicion.