Pediatric Pulmonary Embolism: Diagnostic and Management Challenges.

A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predisposing factors. Computed tomography pulmonary angiography supported by three-dimensional reconstruction was diagnostic. The embolus qualified as massive by conventional anatomical guidelines, but as low risk by more recent functional criteria. Functional assessment has complemented morphologic assessment for risk stratification in adult patients. Such evidence is scarce in pediatrics. The patient underwent surgical embolectomy, followed by prophylactic anticoagulation, without further events. Diagnostic and management challenges are discussed.

Tetralogy of Fallot and anomalous origin of the left pulmonary artery from the ascending aorta associated with absent right internal carotid artery.

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malformation more commonly described with tetralogy of Fallot (TOF). The association of absent right carotid artery with TOF and anomalous left pulmonary artery from the ascending aorta has been rarely reported in the past. This case highlights the need for careful evaluation of the aortic arch and head vessels anatomy in patients with TOF because of the potential neurologic complications after surgery.

Double aortic arch associated with bronchopulmonary sequestration: a rare dual anomaly.

We report two isolated and rare congenital malformations, namely double aortic arch and bronchopulmonary sequestration occurring as coexisting lesions in an infant. Clinical presentation leading up to a detailed anatomic diagnosis, using echocardiography and multidetector computerized tomographic angiographic imaging are described. To our knowledge, our patient is the second case reported with this dual pathology, making it an exceedingly rare occurrence.

Airway management following pediatric cardiothoracic surgery.

OBJECTIVES: (1) Review airway management in pediatric patients undergoing cardiothoracic surgery (CTS); (2) determine the incidence of airway-related complications of CTS in this population. DESIGN: Case series with chart review. Setting Tertiary care children's hospital. Patients Children undergoing CTS over a 4-year period. METHODS: Patients who underwent CTS at a single, tertiary care, children's hospital between June 1, 2007, and May 31, 2011, were retrospectively reviewed; those <18 years who had open CTS were included. Statistical analysis examined relationships of intubation duration, complications, and need for tracheotomy while comparing patient characteristics, comorbidities, and types of surgery. RESULTS: Eight hundred seventy-five primary surgeries in 745 patients met inclusion criteria. Mean postoperative intubation duration was 7.2 days and median 3 days. On univariate analysis, significantly longer postoperative intubation requirements were found in patients younger in age, with congenital comorbidities or prematurity, with preoperative ventilation requirements, and those with early postoperative complications. Multivariate analysis found younger age, presence of congenital comorbidities, preoperative intubation requirements, and early postoperative complications each lengthen ventilation requirements. Four patients developed vocal cord paralysis and 5 developed phrenic nerve palsy. Nineteen patients required tracheotomy. CONCLUSIONS: In this large cohort, CTS in the pediatric population is associated with few long-term or permanent airway-related complications. Patients who are younger in age and those with congenital comorbidities, preoperative ventilation requirements, or early postoperative complications required longer periods of postoperative intubation.

Left atrial myxoma in a child: a challenging diagnosis of a rare lesion.

An eight-year-old child presented with congestive heart failure, blurred vision, and unexplained constitutional symptoms. An echocardiogram demonstrated a giant left atrial mass that obstructed the mitral valve inflow. After excision of the myxoma, the patient had an uneventful recovery.

Double aortic arch and aortopulmonary window: an uncommon presentation in a newborn.

We describe a rare association of aortopulmonary window and double aortic arch in a 1.7-kg newborn who presented with severe respiratory distress. A staged surgical approach was used because of the size of the patient and significant comorbidity. This approach resulted in excellent outcome.

Two-stage repair of truncus arteriosus associated with interrupted aortic arch in critically ill patients.

This report describes 2 newborns with truncus arteriosus associated with an interrupted aortic arch who underwent a 2-stage repair due to poor preoperative condition. A repair of the interrupted aortic arch and ventricular-to-pulmonary artery conduit with a 6-mm Gore-Tex tube was performed as a first stage. Closure of a ventricular septal defect and placement of a right ventricular-to-pulmonary artery homograft conduit were performed electively at 8 months of age.

Development of the pulmonary arteries after the Norwood procedure: comparison between Blalock-Taussig shunt and right ventricular-pulmonary artery conduit.

BACKGROUND: The Norwood-Sano procedure for hypoplastic left heart syndrome (HLHS) has been associated with improved postoperative hemodynamics and outcome. This study compared the effect of a Blalock-Taussig shunt with right ventricular-pulmonary artery (RV-PA) conduit before bidirectional Glenn on the development of the PAs. METHODS: Between January 2000 and June 2007, 42 patients with HLHS underwent bidirectional Glenn: 19 (mean age, 7 +/- 1.5 months) had a Blalock-Taussig shunt (3.5 mm in 15; and 4 mm in 4); 23 patients (mean age, 5 +/- 1 month) had RV-PA conduit (5-mm tube). Patients underwent postoperative cardiac catheterization and echocardiograms. RESULTS: Total PA (Nakata) index was 192 +/- 10 mm2/m2 in the Blalock-Taussig group and 238 +/- 18 mm2/m2 in the RV-PA conduit group (p = 0.03). In the RV-PA conduit patients, the left and right PAs had comparable diameters. In the Blalock-Taussig group, the left PA was smaller than the right (p = 0.02). The mean PA pressure at the end of the operation was 14 +/- 2 mm Hg in the Blalock-Taussig group and 11 +/-1 mm Hg in the RV-PA conduit group (p = 0.06). Persistent pleural effusion occurred in 4 Blalock-Taussig patients (21%) and in 1 (4%) with RV-PA conduit (p < 0.05). Postoperative arterial oxygen saturation was 80% +/- 2% in the RV-PA conduit group and 74% +/- 2% in the Blalock-Taussig group (p < 0.05). CONCLUSIONS: The Norwood procedure with RV-PA conduit may have favorable effects on the development of the PA due to even distribution of pulmonary blood flow.

Reimplantation of anomalous left coronary artery from the pulmonary artery without mitral valve repair.

BACKGROUND: Early establishment of a two-coronary artery system has become the standard surgical approach in patients with anomalous origin of the left coronary artery from the pulmonary artery. Improved surgical outcome is related to better perioperative management and surgical techniques. The need for mitral valve repair is still controversial, however. We report our long-term results with aortic implantation of the left coronary artery. METHODS: Between January 1992 and July 2005, 23 patients who had severe left ventricular dysfunction and mitral insufficiency underwent aortic implantation of the left coronary artery. Mean age and weight were 5 +/- 3 months (range, 2 to 14 months) and 5 +/- 1.5 kg (range, 3.2 to 7 kg). Repair was accomplished by creating a wide anastomosis between the left coronary artery and aorta that was augmented with autologous pericardium to avoid tension and distortion of the anastomosis. None of the patients had concomitant mitral valve repair. RESULTS: There were no operative or late deaths. One patient required extracorporeal membrane oxygenation for 86 hours and another for 100 hours because of sustained ventricular tachycardia and respiratory insufficiency. A younger age (<6 months) was associated with a longer stay in the intensive care unit (p = 0.04). During a mean follow-up of 78 +/- 30 months (range, 6 to 156 months) all patients were in the New York Heart Association functional class I. Serial echocardiograms showed complete recovery of left ventricular function in all patients within 6 months. Mitral valve function was normal in 17 patients and mildly impaired in 6. The mean shortening fraction increased from 0.2 +/- 0.05 preoperatively to 0.43 +/- 0.07 postoperatively (p = 0.03), and left ventricular end-diastolic dimension decreased from 44 +/- 7 mm preoperatively to 29 +/- 8 mm postoperatively (p = 0.02). CONCLUSIONS: Aortic implantation of the left coronary artery results in complete recovery of left ventricular function and no late mitral valve dysfunction.

Pulmonary atresia, intact ventricular septum, right ventricle-dependent coronary artery circulation, and systemic collateral arteries supplying the left coronary artery.

We present a 14-day-old with pulmonary artery atresia with intact ventricular septum, right ventricle-dependent coronary circulation, a single aortic root coronary ostia (left), congenital collateral arterial supply to the left coronary artery from the left internal mammary artery, and bilateral paravertebral arteries, with obstructive coronary artery lesions.

Effects of controlled antegrade pulmonary blood flow on cardiac function after bidirectional cavopulmonary anastomosis.

BACKGROUND: Bidirectional cavopulmonary anastomosis (BCPA) has been used as an intermediate stage in the treatment of patients with single-ventricle physiology. Leaving additional antegrade pulmonary blood flow has been shown to improve postoperative arterial blood oxygen saturations; however, controversy continues over whether the potential increase in systemic venous pressure is detrimental. We studied the effects of controlled antegrade pulmonary blood flow on cardiac function in patients after BCPA. METHODS: From January 1993 to July 2000, 128 patients underwent BCPA. Mean age at operation was 6.2 +/- 4 months (range 2 to 36 months). In group 1 (n = 72), restricted antegrade pulmonary blood flow was maintained through a native narrowed pulmonary valve or by adjustment of previously placed pulmonary artery band with the goal of maintaining the mean pulmonary artery pressure less than 16 mm Hg. In group 2 (n = 56), BCPA was the only source of pulmonary blood flow. RESULTS: One hospital death (0.8%) occurred. The mean pulmonary artery pressure at the end of the operation was 13 +/- 2 mm Hg in group 1 compared with 12 +/- 2 mm Hg in group 2, a difference that was not significant. Patients in group 1 had higher arterial oxygen saturations (84% +/- 3% compared with 74% +/- 3% in group 2, p < 0.05), and shorter mean hospital stay (9 +/- 3 days compared with 15 +/- 2 days, p < 0.05). Persistent pleural effusion (> 10 days) or late chylothorax occurred in 4 patients from group 1 and 3 from group 2, a difference that was not significant. During a mean follow-up of 36 +/- 10 months no late deaths occurred. The mean oxygen saturation remained higher in group 1, 80% +/- 3% compared with 74% +/- 4% in group 2, and the hematocrit was lower, at 38% +/- 3% compared with 46% +/- 4% (p < 0.05 for both comparisons). Cardiac catheterizations were performed in 68 patients before completion Fontan. Total pulmonary artery (Nakata) index was 263 +/- 34 mm(2)/m(2) in group 1 (n = 40) and 188 +/- 13 mm(2)/m(2) in group 2 (n = 28) (p < 0.05). The mean pulmonary artery pressure and mean ventricular end-diastolic pressure were similar. CONCLUSIONS: Controlled antegrade pulmonary blood flow may have favorable effects on cardiac function for a selected group of patients and does not appear to have adverse effects on subsequent suitability for completion Fontan.

Treatment of persistent chylothorax after Norwood procedure with somatostatin.

A newborn who had undergone Norwood procedure for hypoplastic left heart syndrome developed a voluminous chylothorax that persisted despite weeks of prolonged complete bowel rest, total parenteral nutrition, and effective chest tube drainage. Chest tube output diminished immediately following initiation of intravenous somatostatin, allowing restoration of full enteral feeds and removal of chest tubes within 6 days.