Percutaneous Coil Closure of a Large Left Coronary Artery Fistula in an Asymptomatic Child.

Coronary artery fistulas (CAFs) are rare cardiac anomalies characterized by an abnormal connection between the coronary arteries and either a cardiac chamber or a large thoracic vessel. While the majority of CAF cases are asymptomatic, serious cardiac complications can occur, especially with moderate to large fistulas. We describe a case of a large-sized left coronary artery (LCA) fistula in an asymptomatic 11-year-old who was referred for cardiac evaluation due to a systolic murmur. An echocardiogram revealed a hemodynamically significant fistula arising from the LCA draining into the right ventricle. Diagnostic catheterization confirmed the origin and draining site of the fistula, along with aneurysmal dilation at the end of the fistula. The fistula was successfully closed percutaneously using a two-coil occlusive device, with no complication observed.

Prolonged electrical quiescence after direct current cardioversion for atrial flutter in congenital heart disease.

Elective direct current cardioversion is considered first-line treatment in many cases of atrial flutter and fibrillation. This also is true in the pediatric population. This report describes a case of successful cardioversion that resulted in a very prolonged electrical quiescence.

The use of 3D contrast-enhanced CT reconstructions to project images of vascular rings and coarctation of the aorta.

BACKGROUND: Aortic arch and pulmonary artery anomalies make up a group of vascular structures that have complex three-dimensional (3D) shapes. Tortuosity as well as hypoplasia or atresia of segments of the aortic arch or pulmonary artery makes the conventional two-dimensional (2D) imaging difficult. METHODS: Nine patients with native coarctation or recoarctation and 4 patients with a vascular ring had a CT scan as a part of their clinical evaluation. There were 7 males. The mean age was 11.7 years. (range 19 days to 29 years) The mean weight was 22.7 kg (range 3.3-139.0 kg). The dicom data from contrast CT scans were converted by the Amira software package into a 3D image. The areas of interest were selected. The images were then projected in 3D on a standard video monitor and could be rotated 360 degrees in any dimension. RESULTS: Adequate CT scans and 3D reconstructions were obtained in 12 of 13 patients. There were 85-1,044 slices obtained in the adequate studies. We could not reconstruct a 3D image from a patient's CT scan that had only 22 slices. The anatomy defined by 3D was compared to 2D CT imaging and confirmed by cardiac catheterization or direct visualization in the operating room in the 12 patients with adequate 3D reconstructions. In 5 of 12 patients, 3D reconstructions provided valuable spatial information not observed in the conventional 2D scans. CONCLUSION: We believe that 3D reconstruction of contrast-enhanced CT scans of these complex structures provides additional valuable information that is helpful in the decision-making process.

The use of stereolithographic hand held models for evaluation of congenital anomalies of the great arteries.

Imaging anomalies of the great vessels has traditionally been accomplished using conventional biplane modalities as well as three-dimensional (3D) video displays. Our aim was to review the use of stereolithography to create 3D models to assess coarctation of the aorta and vascular rings. Twelve patients had high-resolution CT scans to evaluate anomalies of the great arteries (coarctation: 9, vascular ring: 3). Ages were 19 days to 29 years and weights were 3.3 to 139 kg. Digital dicom data from each scan were converted by a commercially available software package into a 3D digital image. The area of interest was selected and the image was exported to a 3D stereolithographic printer to create a 3D model. The models were then evaluated and the results compared to catheterization and surgical findings. All models accurately displayed the pathology investigated. All 3 of the vascular ring models correlated with surgical findings (double arch: 2, pulmonary sling: 1). Models of aortic coarctation allowed clear depictions of discrete narrowing as well as arch hypoplasia and tortuosity. Stereolithography can create realistic 3D models that accurately display aortic pathology and add important additional information, which may have implications regarding surgical and transcatheter interventions and may also be useful teaching tools for parents and students.

Brain-type natriuretic peptide in the diagnosis and management of persistent pulmonary hypertension of the newborn.

OBJECTIVE: The diagnosis of persistent pulmonary hypertension (PPHN) can often be difficult to make, especially in a clinical setting in which pediatric echocardiography is not readily available. A noninvasive test that could differentiate PPHN from other cardiorespiratory disease would be very useful in the early management of the disease, because it would allow rapid identification of those infants at greatest risk of requiring the services of a level 3 nursery. Brain-type natriuretic peptide (BNP) is an endogenous peptide hormone secreted by the cardiac ventricles in response to increased wall stress and related ventricular filling pressures. The purpose of this study was to determine if BNP levels are elevated in newborns with PPHN and therefore may be used as a marker for differentiating PPHN from other forms of respiratory disease during the early newborn period. METHOD: We used a prospective cohort design with 3 groups. One group was diagnosed with PPHN by clinical and echocardiographic criteria (PPHN group: n = 15). The second group had been diagnosed with respiratory disease; however, PPHN had been ruled out by having no evidence of elevated pulmonary pressure by echocardiography (RD group: n = 17). The third group had no respiratory disease and was breathing room air (RA group: n = 15). BNP levels were measured with a point-of-care fluorescence immunoassay at various time intervals between birth and 150 hours of life. RESULTS: There were no differences between groups for birth weight, gestational age, gender, race, Apgar scores at 1 minute, or age at time of initial blood sampling. Initial BNP levels (pg/mL) were elevated in the PPHN group relative to both the RA and RD groups (median [25%, 75%]: PPHN group = 1610 [1128, 1745]; RD group = 132 [76, 327]; RA group = 248 [127, 395]). There was no difference in the initial BNP level between the RA and RD groups. BNP levels remained elevated in the PPHN group over both groups for the first 4 days of life. BNP levels correlated with the gradient of the tricuspid regurgitation jet and with the ratio of tricuspid regurgitation jet gradient to mean blood pressure. BNP levels were not affected by administration of dopamine or dobutamine. BNP weakly correlated with the oxygenation index but not with the alveolar-arterial oxygenation gradient. CONCLUSIONS: Our findings indicate that BNP levels are elevated in infants with PPHN but not in infants with other forms of respiratory distress not associated with PPHN. Elevated BNP levels in term or near-term infants with respiratory distress should increase the suspicion of PPHN. Serial determination may also be helpful in monitoring the clinical course of such infants.

Use of small stents for rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect.

Branch pulmonary artery stenosis frequently occurs in pulmonary atresia with ventricular septal defect (PA/VSD). Balloon dilation alone is often unsuccessful in patients with severely hypoplastic pulmonary arteries with residual stenoses after surgical repair. In an attempt to promote distal pulmonary artery growth, 17 stents were placed in 12 severely stenotic pulmonary artery lesions in 10 patients with PA/VSD. All had prior surgery, including pulmonary artery repair, right ventricle to pulmonary artery homograft, and, in 6 of 10, closure of VSD. Median age at stent placement was 16.8 months (range, 13.2-56). Stents were placed using 3.0, 3.5, or 4.0 mm balloons in all but one lesion, in which a 7 mm balloon was used. Following stent placement, there was an increase in the lesion diameter from 1.5 to 3.4 mm (P < 0.05) and an increase in flow to the affected lung from 27% to 34% (P < 0.05). Repeat catheterization 2 to 6 months after stenting in six patients revealed complete occlusion in two of eight lesions. In the other six vessels, there was an increase in distal vessel diameter from 2.96 to 3.94 mm (P < 0.05) even though four had severe restenosis requiring restenting. Two patients underwent surgical pulmonary artery reconstruction and stent removal because of adequate distal vessel growth. Stenting of hypoplastic pulmonary arteries in PA/VSD results in immediate improvement in vessel size and blood flow. Stent restenosis is common although distal vessel growth can be achieved. Stenting of these lesions should be reserved only for those patients unresponsive to other interventions.