Ductus arteriosus: more than just the patent ductus arteriosus.

The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions. These lesions can impact the systemic circulation, the pulmonary circulation or the airway and include coarctation of the aorta, ductal origin of the pulmonary artery and vascular rings.

First antenatally confirmed case of arterial tortuosity syndrome.

Arterial tortuosity syndrome (ATS) is a rare, autosomal recessive, connective tissue disorder. It predominantly involves the arterial tree with clinical features reflecting the systems involved. There have been few cases of ATS suspected during antenatal screening ultrasound in high-risk families, but none confirmed. We present the first case of ATS confirmed antenatally in the fetus with cascade testing, detecting the disease in the mother as well.

Acute respiratory distress secondary to severe compression of the left main bronchus by the ductus arteriosus.

Two newborn infants presented with acute respiratory distress. In both cases, the left lung was opaque, hyperinflated, and associated with a rightward shift of the mediastinum. A diagnosis of retained fetal fluid secondary to vascular compression of the left bronchus by the ductus arteriosus was made by combining various imaging methods including chest radiograph, computed tomography (CT), and echocardiography. Although the initial chest radiographs were similar, the mechanisms of obstruction were different. The imaging emphasizes the importance of CT angiography to understanding the three-dimensional relationships resulting in bronchial compression.

A case of horseshoe lung and complex congenital heart disease in a term newborn.

We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.

Clipped tube fenestration after extracardiac Fontan allows for simple transcatheter coil occlusion.

BACKGROUND: Expensive devices are increasingly used to close a patent fenestration after a modified Fontan operation. We report our 5-year institutional experience of clipped tube fenestration after extracardiac Fontan operation, which allows for simple transcatheter coil occlusion. METHODS: We retrospectively reviewed 30 children, median age of 4.0 years (range, 2.4 to 8.8 years) who underwent extracardiac Fontan operation between May 1996 and May 2001, and were fenestrated using a 4- to 8-mm diameter clipped tube graft. RESULTS: Ten children had a patent fenestration occluded by transcatheter placement of 15 detachable coils (5- to 8-mm diameter). Aortic oxygen saturations increased on average by 5.5% (2% to 14%) and mean pressures in the Fontan circuit by 2.5 mm Hg (0 to 3 mm Hg). Four had immediate complete occlusion angiographically and 6 had trivial residual shunt, but complete occlusion by echocardiography at follow-up. There have been no immediate complications, late coil embolizations, thromboembolic events, or documented hemolysis within a follow-up after coil implantation of 1.7 years (0.4 to 4.5 years). Spontaneous fenestration closure was documented in 8 patients at cardiac catheterization and 9 patients by echocardiography with consistent improvement in resting transcutaneous oxygen saturation. Two children with a patent fenestration have been considered inappropriate for closure, and there was one early surgical death. There have been no complications related to the tube fenestration modification within a follow-up postoperation of 2.6 years (0.1 to 5.5 years). CONCLUSIONS: Clipped tube fenestration after extracardiac Fontan operation is a useful surgical modification that allows for simple transcatheter coil occlusion.

Balloon angioplasty of right ventricular outflow tract conduits.

Palliation of complex congenital heart disease, requiring reconstruction of the right ventricular outflow tract (RVOT), is standard practice. Survival of the homograft is a limiting factor. We examined the role of balloon angioplasty (BAP) in prolonging conduit life. Twelve patients underwent 15 BAP procedures between February 1989 and October 1997. The median age at conduit insertion was 28 months with detection of a significant echo gradient 42 months later. Calcification of homografts, with attendant obstruction and valve dysfunction, was present in all patients. BAP was performed within 1 month of echocardiography and reduced the gradient from a median of 57 to 38 mmHg (p < 0.0005). Echocardiographic follow-up showed persistent gradients (median 68 mmHg) and 11/12 patients went on to conduit replacement after BAP. Only one patient had replacement deferred as a result of BAP. Complications requiring intervention occurred in 20% of the procedures and included bleeding and an unusual balloon fracture. Although BAP can reduce the pressure gradient across the RVOT conduit, the effect is transient and the delay of surgery is not due to improved hemodynamic function. Approximately 10% of cases will benefit from BAP alone, but given the high rate of complications, we do not recommend this procedure as a means of prolonging conduit life.

The evolution of ductus arteriosus treatment.

The treatment of patent ductus arteriosus (PDA) has evolved over the years. We reviewed 231 non-premature children (group 1) undergoing surgical closure of a PDA between January 1985 and December 1997, and 30 children (group 2) undergoing transcatheter closure from May 1995 to December 1998. The median age and weight at operation in group 1 were 13 months (range, 0.5-174 months) and 9.5 kg (range, 1.9-49.7 kg), respectively. There was one intra-operative death (0.4%) secondary to hemorrhage. Immediate extubation was performed in 208 patients (90%). Intra-operative chest tube use decreased from 73.3% to 10% between the 1985-88 and 1996-97 periods (P < 0.001). Postoperative pneumothoraces occurred in 33/131 (25%) patients with only one patient (0.7%) requiring drainage. Eleven patients had complications including wound infection in four, vocal cord paralysis in three, and left pulmonary artery stenosis in one. The median length of stay (LOS) was 5 days (range, 2-43 days). Follow-up echocardiogram was performed in 146/230 patients (63%) and revealed a residual PDA in six (4%); two being re-ligated, two remaining clinically insignificant, and two spontaneously resolved at 7 and 28 months follow-up. The remaining 84 patients had no clinical signs of a residual PDA. In group 2, where a transcatheter coil occlusion technique was used, the median age and weight at procedure were 31 months (range, 9-320 months) and 14.9 kg (range, 9-69.7 kg), respectively. Vascular complications occurred in four patients (13.3%). One patient developed hemolysis and hemoglobinuria requiring hospital admission. Four patients required a second intervention. At the most recent echocardiographic assessment, four patients (13.3%) had a residual PDA.

Coil occlusion of the patent ductus arteriosus: lessons learned.

PURPOSE: To review the clinical outcomes of catheter-directed coil occlusion (coil occlusion) of persistently patent ductus arteriosus (PDA) at a pediatric tertiary care hospital. METHODS: A retrospective review of all patients referred to the Cardiac Catheterization Laboratory for coil occlusion at our institution was performed. Twenty-one consecutive patients (12 female) underwent coil occlusion and follow-up between May 1995 and December 1997. We undertook PDA occlusion if: (a) the PDA narrowed to less than 4 mm on echocardiogram and (b) the minimum body weight was approximately 10 kg. Standard right and retrograde left heart catheterization was performed, followed by coil occlusion. Color-flow mapping (CFM) was used intra-procedurally to confirm occlusion of the PDA with a follow-up study several weeks later. RESULTS: The median age and weight of the patients were 33 months and 13.2 kg, respectively. Fourteen patients received one coil, with six requiring a second coil and one requiring multiple coils. Initial follow-up was at a median of 2.4 months. At latest follow-up, 2 patients still have persistent flow at the ductal level. The coils were deployed without complication or embolization. CONCLUSIONS: A review of our first 21 cases demonstrated three important lessons: (1) the maximum diameter of the PDA suitable for coil occlusion is approximately 3 mm; (2) CFM must show complete obliteration of flow in the catheterization lab in order to ensure occlusion of the PDA at follow-up; and (3) the Jackson detachable system allows for precise placement of the coil, often within another coil.

Early and intermediate-term complications of self-expanding stents limit its potential application in children with congenital heart disease.

OBJECTIVES: We report on the early and intermediate-term follow-up results of self-expanding Wallstent (Schneider, Switzerland) implanted in children with congenital heart disease. BACKGROUND: The inherent shortcomings of balloon-expandable stents prompted the trial of an alternative stent. METHODS: Twenty patients underwent 22 implantations of 25 self-expanding Wallstents between December 1993 and June 1997 in two institutions. The mean age and weight were 10.8+/-4.5 years and 30.5+/-14.2 kg, respectively. The patients were divided into two groups: 1) Group I comprised 17 patients with pulmonary arterial stenoses, 2) Group II comprised four patients with venous stenoses (one belonged to both groups). Sixteen patients underwent recatheterization at a median of 5.8 months (range 0.5 to 31, mean 8.1 months) after stenting. Hemodynamic and angiographic changes after the interventional procedures and complications were documented. RESULTS: All the stents were successfully deployed in the intended position. In Group I, the narrowest diameter of the stented vessel increased from 4.1+/-1.5 to 8+/-2 mm (95% increase, p < 0.0001) while the systolic pressure gradient across decreased from 24.6+/-15.8 to 12.1+/-11.4 mm Hg (51% decrease, p = 0.001). In Group II, the dimensional changes of the narrowest segment increased from 4.3+/-0.5 to 7.5+/-0.4 mm (75% increase, p = 0.003), and the pressure gradient reduced from 5.0+/-2.9 to 0.9+/-1.0 mm Hg (82% decrease, p = 0.04) across the stented venous channel. Distal migration of two optimally positioned stents occurred within 24 h of implantation. At recatheterization, significant neointimal ingrowth (>30% of the expanded diameter) was noted in 7 (28%) of the 25 implanted stents. This responded poorly to balloon dilation. Predisposing factors for the neointimal ingrowth included stents of smaller diameter (<9 mm) and longer period after implantation. CONCLUSIONS: Self-expanding Wallstent could be deployed easily and safely to relieve vascular stenoses in children. The complications of distal migration, significant neointimal ingrowth and its unyielding design to overdilation limit its application to this patient group.

Extracardiac Fontan operation with tube fenestration allowing transcatheter coil occlusion.

A fenestration may improve the immediate postoperative course after a Fontan procedure by preserving the cardiac output. We describe a simple and safe technique of fenestration amenable to coil occlusion, which can be carried out in most cardiac catheterization laboratories.

Cine phase-contrast MR to assess portal blood flow in a 10-year-old girl with abdominal aortic coarctation: a case report.

We report the case of a 10-year-old girl with repaired abdominal aortic coarctation in whom chronic mesenteric ischemia was clinically suspected. Cine phase-contrast magnetic resonance (MR) was used to determine the difference between fasting and postprandial portal blood flow. Fasting flow rates in the portal vein were normal. After a meal, blood flow in the portal vein increased 226% over the fasting state, showing normal augmentation. To the best of our knowledge, this is the first application of this technique to a pediatric setting.

Dilatable prosthesis for banding the main pulmonary artery: human clinical trials.

The objectives of this trial are to assess the safety and effectiveness of the prosthesis and to establish the ability of the dilatable band to provide a nonsurgical option for dilatation. Forty-six patients received dilatable bands. All had congenital heart defects requiring banding of the main pulmonary artery. Dilatation was performed on 7 patients. This was successful in 6 and uncomplicated in all. In one patient the dilatable band was adjusted too tightly at implantation. In one patient the band was adjusted to loosely. In 7 patients the dilatable band was placed too distally and partially obstructed the right or left pulmonary artery. Distal migration of the dilatable band after implantation did not occur. One band was distorted during implantation. This did not compromise its function. Surgical sepsis resulted in a mycotic aneurysm and erosion of the pulmonary artery in one patient. Surgical pulmonary arterioplasty was performed in all 18 patients who had total correction and in 11 of the 13 patients who had bidirectional Glenn procedure and Damus-Stanzell connection. There were 13 deaths. None of the deaths were related to the dilatable band. Thirty-two prostheses were surgically explanted readily and completely in 31 patients. Five bands were removed at postmortem examination. Examination of all 37 of the dilatable bands revealed no evidence of wear or damage. Scanning electron microscopy evaluation was conducted on 5 of the explanted devices which had been implanted 158 to 1139 (mean 422) days. No component failure was identified. The dilatable band prostheses was effective and safe and provided a non-surgical option for dilatation.

Splanchnic artery pseudo-aneurysms secondary to blunt abdominal trauma in children.

Traumatic splanchnic artery pseudo-aneurysms are uncommon; only two cases have been reported among the pediatric population. The authors describe their experience with four patients in whom splanchnic artery pseudoaneurysms developed after blunt abdominal trauma. Splenic artery pseudo-aneurysms were found in a 6-year-old boy and an 8-year-old girl after blunt splenic injuries. In both cases, spontaneous thrombosis of the pseudo-aneurysms occurred after a period of observation. Hepatic artery pseudoaneurysms were found in a 7-year-old boy and a 10-year-old girl after major liver lacerations. The boy had successful angiographic embolization of the lesion, but the girl required direct ligation of the pseudo-aneurysm after nearly exsanguinating from acute hemorrhage. All four children have recovered completely, with no long-term sequelae. Traumatic splanchnic artery pseudo-aneurysms are potentially life-threatening complications that can occur after blunt abdominal trauma. The investigation and management of these lesions must be individualized according to the clinical scenario.

Assessment of airways compression by MR imaging in children with aneurysmal pulmonary arteries.

Magnetic resonance imaging (MRI) has an established role in the accurate and non invasive assessment of airways compression by congenital vascular rings and pulmonary artery slings, making angiography of these lesions unnecessary. This role can be broadened to encompass other vascular compressive lesions, as in the two pediatric patients described here with aneurysmal pulmonary arteries of different etiology, one congenital and the other acquired.

Partial splenic embolization in children with hypersplenism.

As an alternative to surgical splenectomy, partial splenic embolization was performed in seven children for hypersplenism manifested by splenomegaly, thrombocytopenia, leukopenia, and erythrocyte hemolysis. Within a few days, platelet and leukocyte counts rose significantly in all patients and were maintained in six of seven patients during a follow-up period of 9 to 69 months. Spleen size and abdominal distention also decreased significantly in all children. There were no infectious complications.