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The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important for complete anatomical delineation and planning for treatment. Preoperative imaging used for assessment of the main pulmonary artery (its size, valve, and confluence), aortopulmonary collaterals (its origin, insertion, course, and size), presence of patent ductus arteriosus, other sources of collaterals as bronchial and coronary arteries, and pattern of pulmonary arborization. Imaging can detect associated aortic, pulmonary venous and coronary anomalies, and other congenital heart disease. Postoperative imaging after unifocalization and stent is for assessment of patency, stenosis, and occlusion of stent or perivascular lesions as seroma.

Rationale: Page kidney is an uncommon condition that hypertension occurs secondary to microvascular ischemia and alternation of small-vessel hemodynamics due to external compression of renal parenchyma and activation of the renin-angiotensin-aldosterone system. There are no specific guidelines for the management of Page kidney in the literatures., Patient Concerns: A 17-year-old teenager who had Fontan procedure for tricuspid and pulmonary atresia in early childhood suffered from sudden onset of severe left flank pain during cardiac catheterization procedure. Left renal artery active bleeding with renal parenchyma compression in association with renin-mediated hypertension led to the diagnosis of Page kidney., Diagnoses: Page kidney was diagnosed in this case., Interventions: Urgent embolization was performed to treat left renal artery active bleeding. Because of decreased renal function with elevation of serum creatinine, inadequate blood pressure control with antihypertensive medication, and poor renal blood flow of left kidney, open drainage of perirenal hematoma was done 5 days after catheterization procedure., Outcomes: After the operation, glomerular filtration rate improved immediately, and left flank pain and hypertension resolved at discharge., Lessons: The choice of the therapies for Page kidney depended on the clinical presentation of each case. This case pointed out the significance of renoscintigraphy and surgery in the management of Page kidney.

Objectives: The objectives of this review are to discuss the anatomy, pathophysiology, clinical course, and current treatment strategies for pulmonary atresia with intact ventricular septum, pulmonary stenosis, and Ebstein's anomaly., Data Source: MEDLINE and PubMed., Conclusions: Considerable advances have been made in management strategies for these complex congenital heart lesions, which have led to improved outcomes.

The objective of this study was to investigate whether the α agonist dexmedetomidine has the ability to attenuate hypoxemia in pediatric patients undergoing palliative pulmonary artery reconstruction.From January 2009 to January 2013, a total of 25 pediatric patients with Tetralogy of Fallot, pulmonary atresia (ventricular septal defect), or persistent truncus arteriosus (I) were enrolled in our study. Due to hypoplastic pulmonary arteries, all patients received palliative pulmonary artery reconstruction. During the perioperative period, they were allocated to receive either dexmedetomidine (bolus dose of 0.3 μg/kg followed by an infusion of 0.2-0.3 μg/kg/h, n = 15) or control drug (n = 10) intravenously. Any desaturation was recorded. Heart rate, mean arterial pressure, pulse oximetry, and arterial blood gas parameters were measured during the perioperative period.There were no significant differences between the groups in hemodynamic variables. The arterial oxygen saturation and arterial blood gas parameters increased in the dexmedetomidine groups (P < 0.05).These findings suggest that the injection of dexmedetomidine can attenuate hypoxemia during palliative pulmonary artery reconstruction in pediatric patients.

Objective: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs), who undergo unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to identify risk factors that are associated with prolonged mechanical ventilation in this population., Design: We collected preoperative and operative data from medical records and postoperative data prospectively. Mechanical ventilation beyond postoperative day 5, which was greater than the 50th percentile for the subjects enrolled, was considered prolonged. Risk factors were analyzed using multiple logistic regression, independent samples t test, Fisher's exact test, and Kruskal-Wallis test. Enrollment occurred over a 20-month period between May 2009 and January 2011., Setting: Lucile Packard Children's Hospital (Palo Alto, CA)., Patients: All patients with TOF/PA/MAPCAs presenting for unifocalization or pulmonary artery revision procedures were eligible, including those with additional structural cardiac abnormalities requiring surgical intervention. We excluded patients with single-ventricle cardiac anatomy and preoperative respiratory failure or infection., Measurements and Main Results: We enrolled 35 consecutive patients undergoing 37 procedures over the study period. One patient was excluded for single-ventricle anatomy. There were 12 cases (32%) of prolonged mechanical ventilation. Delayed sternal closure was the only risk factor associated with prolonged mechanical ventilation (p = 0.01). Age, weight, cardiopulmonary bypass time, 22q11 microdeletion, postoperative fluid balance, bronchospasm, and nonrespiratory infection were not significantly associated with prolonged mechanical ventilation. Respiratory complications occurred in both groups, and patients with pneumonia were more likely to have a prolonged course (p = 0.03). There was no significant association between the type of surgery performed and duration of mechanical ventilation., Conclusions: Prolonged postoperative respiratory failure in children undergoing surgery for TOF/PA/MAPCAs was independently associated with delayed sternal closure. Respiratory complications occur after unifocalization surgery, and pneumonia is associated with prolonged mechanical ventilation. Our pilot study suggests that clinical features common in this patient population, such as bronchospasm and 22q11 microdeletion, were not associated with more postoperative respiratory failure.

Background: Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot., Methods and Results: A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%)., Conclusions: Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

We report the case of a 14-year-old boy affected by Tetralogy of Fallot and pulmonary atresia, who had already undergone multiple surgical procedures. He had later developed a steno-insufficiency of the pulmonary bioprosthesis. We performed a percutaneous pulmonary valve implantation (PPVI), which is a minimally invasive treatment, as effective as conventional valve surgery and associated with less morbidity and mortality. Up to now PPVI has been carried out in patients with chronic pulmonary vascular resistance (PVR) in the presence of a right ventricular outflow tract (RVOT) patch by prior deployment of an intravascular stent as an artificial conduit that makes subsequent valve implantation possible. The peculiarity of our procedure was that we implanted the valve directly on to the annulus of the bioprosthesis (valve-in-valve), without performing a prestenting of the prosthetic valve.

Background: Transcatheter pulmonary valve placement is an emerging therapy for pulmonary regurgitation and right ventricular outflow tract obstruction in selected patients. The Melody valve was recently approved in the United States for placement in dysfunctional right ventricular outflow tract conduits., Methods and Results: From January 2007 to August 2009, 136 patients (median age, 19 years) underwent catheterization for intended Melody valve implantation at 5 centers. Implantation was attempted in 124 patients; in the other 12, transcatheter pulmonary valve placement was not attempted because of the risk of coronary artery compression (n=6) or other clinical or protocol contraindications. There was 1 death from intracranial hemorrhage after coronary artery dissection, and 1 valve was explanted after conduit rupture. The median peak right ventricular outflow tract gradient was 37 mm Hg before implantation and 12 mm Hg immediately after implantation. Before implantation, pulmonary regurgitation was moderate or severe in 92 patients (81% with data); no patient had more than mild pulmonary regurgitation early after implantation or during follow-up (>or=1 year in 65 patients). Freedom from diagnosis of stent fracture was 77.8+/-4.3% at 14 months. Freedom from Melody valve dysfunction or reintervention was 93.5+/-2.4% at 1 year. A higher right ventricular outflow tract gradient at discharge (P=0.003) and younger age (P=0.01) were associated with shorter freedom from dysfunction., Conclusions: In this updated report from the multicenter US Melody valve trial, we demonstrated an ongoing high rate of procedural success and encouraging short-term valve function. All reinterventions in this series were for right ventricular outflow tract obstruction, highlighting the importance of patient selection, adequate relief of obstruction, and measures to prevent and manage stent fracture. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

Background: The objective was to determine if intraoperative pulmonary artery (PA) flow studies after complete unifocalization correlate with postrepair hemodynamics for pulmonary atresia (PA), ventricular septal defects (VSD), and major aortopulmonary collaterals., Methods and Results: Twenty patients (median age, 8 months; weight, 7.9 kg) underwent unifocalization between 2003 and 2008. A functional PA flow study was achieved by cannulating the unifocalized central PA before intracardiac repair and increasing flow incrementally to 2.5 L/min per m(2). Mean PA pressure (mPAP) was measured. The intent was to close the VSD for a mPAP of <30 mm Hg. Right ventricular systolic pressure (RVSP) and systemic systolic pressure were recorded. Total incorporated pulmonary segments, pulmonary segment artery ratio (ratio of incorporated segments to 18), and total neopulmonary artery index (the sum of major aortopulmonary collaterals and native PA index) were calculated. The VSD was successfully closed in 18 patients (90%). One attempted closure required an intraoperative fenestration. The study mPAP correlated with RVSP (rho=0.72; P=0.0027) and RVSP/systemic systolic pressure (rho=0.67; P=0.0063). Total neopulmonary artery index had a nonsignificant negative correlation with RVSP (rho=-0.42; P=0.079). Total incorporated pulmonary segments and pulmonary segment artery ratio were not correlated. Flow study mPAP had the highest accuracy in predicting successful VSD closure: area under the receiver-operator curve (0.83) versus total neopulmonary artery index (0.42), pulmonary segments (0.35), and pulmonary segment artery ratio (0.33)., Conclusions: The intraoperative pulmonary flow study predicted postoperative physiology significantly better than did standard anatomic measures. Conventional measures should be used with caution when determining the possibility for complete repair.

Echocardiographic diagnosis of absent pulmonary valve syndrome and muscular ventricular septal defect was made in a fetus of gestational age 25 weeks referred for marked dilation of the right ventricle at obstetric ultrasound examination. Delivery was planned in a tertiary-level center. The neonate became severely symptomatic for respiratory distress and heart failure during the second day of life. His clinical condition dramatically improved after surgical closure of a large ductus arteriosus. The child is still asymptomatic 30 months later. In the minority of cases with absent pulmonary valve not associated with tetralogy of Fallot, irrespective of the presence of muscular ventricular septal defect, early closure of the ductus may be crucial to improve hemodynamic conditions and postpone surgical correction.

During the last 20 years, pediatric cardiac surgery has been characterized by important changes, with reductions in surgical mortality and the achievement of complete repair at an earlier age, thus avoiding multiple procedures and strongly ameliorating the global outcome of these patients. In this review, we describe the actual trends in the surgical treatment of cardiac malformations. We analyze two groups of patients: in the first group (septal defects, tetralogy of Fallot, transposition of the great arteries, aortic stenosis and coarctation) the indications are well established and the goal is represented by a lessening of the surgical trauma and post-operative morbidity, with stable results in the follow-up. In the second group (univentricular heart, pulmonary atresia and intact ventricular septum, double discordance, conduit, hypoplastic left heart syndrome), the lesions are still considered complex and submitted to ongoing experimental and clinical research, in order to improve the post-surgical history of these diseases.

Background: This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease., Methods and Results: Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively. Diagnoses were pulmonary atresia and ventricular septal defect in 11, tetralogy of Fallot in 6, and other forms of pulmonary stenosis or atresia in 6. Median age and weight at diagnosis were 9 years (range, 6 days to 43 years) and 24 kg (range, 2.6 to 60 kg). Fourteen patients had had a previous surgery. The occluded pulmonary artery was visualized at angiography by wedge injection or injection into the collateral circulation. The left pulmonary artery was occluded in 20 patients and the right pulmonary artery in 3. Criteria for reconstruction were estimated duration of occlusion <6 months and ratio of occluded to contralateral artery >0.2. Twelve patients fulfilled these criteria and underwent pulmonary artery reconstruction at a mean interval of 2 months (range, 6 days to 6 months) from evidence of occlusion. Six patients had pericardial patch reconstruction, 3 terminoterminal anastomosis, 2 thrombectomy, and 1 a Blalock-Taussig shunt. There was 1 late death. At a median follow-up of 4 years (2 months to 5 years), all patients underwent cardiac catheterization: in 8 patients the reconstructed artery was patent, in 3 reoccluded. Hypoplasia of the occluded artery was reversed in 6 patients., Conclusions: Our data show that in selected patients, reconstruction of an occluded pulmonary artery can restore pulmonary vascularization and reverse hypoplasia. Strict surveillance is mandatory to prevent pulmonary artery loss.

Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve., Methods and Results: Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1(1/2)-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time., Conclusions: Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle-dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.

Purpose: The purpose of this work was to evaluate electron beam CT (EBCT) for the noninvasive assessment of modified Blalock-Taussig (BT) shunt patency in patients with pulmonary atresia., Method: Five infants and children with pulmonary atresia and modified BT shunts underwent contrast-enhanced EBCT. Modified BT shunts from the subclavian artery to the pulmonary artery were performed to improve the pulmonary blood flow. Electrocardiogram (ECG)-triggered EBCT was obtained with a 100 ms exposure, 3 mm section thickness, and 2 mm table feed after intravenous administration of contrast material. Three-dimensional (3D) or maximum intensity projection (MIP) EBCT images were compared with conventional angiography. The visibility of modified BT shunts was graded and recorded with use of a four-point scale., Results: Satisfactory visualization was achieved in both 3D and MIP EBCT images to evaluate modified BT shunt patency., Conclusion: Contrast-enhanced 3D or MIP EBCT imaging with ECG trigger may be used as an effective substitute to evaluate modified BT shunts with low radiation dose exposure.

Background: While unifocalization techniques have improved the treatment options in patients with pulmonary atresia, ventricular septal defect (PA-VSD), and major aortopulmonary collaterals (MAPCAs), severe airflow limitation contributes to significant early postoperative morbidity and mortality. Although this has been attributed to bronchospasm, characteristically it is refractory to bronchodilators, suggesting that other mechanisms may play a role., Methods and Results: The clinical course and preoperative angiograms of patients who underwent unifocalization were reviewed. Patients who developed airflow limitation early after surgery underwent fiberoptic bronchoscopy. In addition, the anatomy of the MAPCAs was examined in 14 heart-lung blocks from patients with PA-VSD. Twenty-two procedures were performed in 16 children. Three developed marked airflow limitation early after surgery, necessitating prolonged high-pressure ventilation. Bronchoscopy demonstrated tracheobronchial epithelial necrosis in 2 and signs of tracheobronchial ischemia in the third. Two were successfully extubated after 15 and 16 days, but the third died after 57 days of ventilatory support. Review of the preoperative angiograms demonstrated an extensive peribronchial arterial supply arising from a MAPCA in 1 of the patients who developed severe airway necrosis after unifocalization. This was also obvious in a second patient, but the MAPCA was not included in the unifocalization. In 7 autopsy specimens, MAPCAs contributed to a peribronchial or peritracheal vascular network. Dissection of the distribution of these branches in 2 specimens revealed extensive intrapulmonary peribronchial anastomoses., Conclusions: Airflow limitation early after unifocalization is related to airway ischemia resulting from interruption of the tracheobronchial blood supply during mobilization of MAPCAs.

Background: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients., Methods and Results: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients., Conclusions: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

Background: Frequently the definitive operation for patients with a right ventricle (RV) that is too small to support full cardiac output is a modified Fontan operation. However, other surgical options exist that incorporate a small RV in the atriopulmonary pathway when biventricular repair is not feasible because of RV or tricuspid valve hypoplasia. The risks and benefits of these options have not been well defined., Methods and Results: Between 1988 and 1993, 8 patients (6 with pulmonary atresia and intact ventricular septum and 2 with tricuspid valve stenosis and RV hypoplasia) underwent a cavopulmonary connection, which allowed right atrial blood to flow either to the pulmonary artery via the RV or directly via the cavopulmonary anastomosis. Age at surgery ranged from 1.5 to 9 years. The proximal right pulmonary artery was ligated in 5 patients, and the atrial septal defect was closed during the same procedure in 7 of the 8 patients. The echocardiographic right ventricular-left ventricular volume ratio ranged from 9% to 25%, and tricuspid valve z-scores ranged from 0 to -4. There were no deaths at a median follow-up of 24 months (range, 7 to 61 months). Mild exertional limitation was evident in only one patient. Postoperative echocardiograms demonstrated pulsatile systolic flow across the RV outflow tract in 5 patients and low-velocity diastolic-systolic flow in a sixth patient with extreme tricuspid valve hypoplasia. At postoperative cardiac catheterization (6 patients) right atrial mean pressures ranged from 7 to 13 mm Hg and mixed venous saturations from 62% to 70%., Conclusions: Right atrial decompression via a superior vena cava-to-pulmonary artery anastomosis allows incorporation of a small RV into the pulmonary circulation and closure of the atrial septum, with excellent results to date.

The Blalock-Taussig anastomosis (B-T shunt) has been regarded as an unsatisfactory procedure for infants and newborns with severe pulmonary oligemia. With use of microsurgical technique, B-T shunts were constructed in 11 infants under 6 months of age, six of whom were less than 1 month old (2 to 30 days). Weights ranged from 2.4 to 5.6 kg. Diagnoses included transposition of the great arteries (4), tetralogy of Fallot (4), pulmonary arterial atresia (2), and Taussig-Bing syndrome (1). There was no operative mortality; perioperative morbidity included paralysis of the diaphragm (2), Horner's syndrome (1), superficial wound dehiscence (1), congestive heart failure (1), and prolonged respiratory failure requiring ventilation assistance for more than 5 days (2). There has been one late death in the group at 2 months post-shunt. All surviving patients have had satisfactory palliation of cyanosis and hypoxemia. In view of the pulmonary artery scarring and kinking that follows the Waterston and Potts shunts, it is concluded that the Blalock-Taussig shunt is a reliable and preferable technique for palliation of pulmonary oligemia in infants and newborns.