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Byline: Adriano Carotti, Roberto M. Di Donato, Cosimo Squitieri, Paolo Guccione, Glauco Catena Abstract: Background: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. Methods: Since January 1994, 15 consecutive patients (aged 64 [+ or -] 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm.sup.2/m.sup.2 underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm.sup.2/m.sup.2 underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. Results: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P = .006) within 22 [+ or -] 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 [+ or -] 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 [+ or -] 12 months), all patients are free of symptoms, requiring no medications. Conclusion: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results. (J Thorac Cardiovasc Surg 1998;116:914-23) Article History: Received 8 May 1998; Revised 16 July 1998; Revised 4 August 1998; Accepted 6 August 1998 Article Note: (footnote) [star] Sponsor: Aldo R. CastaA[+ or -]eda, MD, [star][star] From the Divisions of Pediatric Cardiac Surgery,a Pediatric Cardiology,b and Pediatric Cardiac Anesthesia,c Department of Pediatric Cardiology and Cardiac Surgery, Bambino GesA[sup.1] Hospital, Rome, Italy., a Address for reprints: Adriano Carotti, MD, Department of Pediatric Cardiology and Cardiac Surgery, Ospedale Bambino Gesu, Piazza S Onofrio, 4, 00165 Rome, Italy., aa 12/6/93657

Specific types and subtypes of cardiac defects have been described in children with 22q11.2 deletion syndrome as well as in other genetic syndromes. The conotruncal heart defects occurring in patients with 22q11.2 deletion syndrome include tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic arch, isolated anomalies of the aortic arch, and ventricular septal defect. These conotruncal heart defects are frequently associated in this syndrome with additional cardiovascular anomalies of the aortic arch, pulmonary arteries, infundibular septum, and semilunar valves complicating cardiac anatomy and surgical treatment. In this review we describe the surgical anatomy, the operative treatment, and the prognostic results of the cardiac defects associated with 22q11.2 deletion syndrome. According to the current literature, in patients with tetralogy of Fallot with/without pulmonary atresia and truncus arteriosus, in spite of the complex cardiac anatomy, the presence of 22q11.2 deletion syndrome does not worsen the surgical prognosis. On the contrary in children with pulmonary atresia with ventricular septal defect and probably in those with interrupted aortic arch the association with 22q11.2 deletion syndrome is probably a risk factor for the operative treatment. The complex cardiovascular anatomy in association with depressed immunological status, pulmonary vascular reactivity, neonatal hypocalcemia, bronchomalacia and broncospasm, laryngeal web, and tendency to airway bleeding must be considered at the time of diagnosis and surgical procedure. Specific diagnostic, surgical, and perioperative protocols should be applied in order to provide appropriate treatment and to reduce surgical mortality and morbidity. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:35–42.

Based on the revolutionary concept of the dispensable right ventricle, the Fontan operation stands as the extreme example of physiologic repair in pediatric cardiac surgery, serving as the common surgical denominator for the entire spectrum of functionally univentricular conditions. Also called ortho-terminal repair, it makes allowance for a circulatory arrangement that is in-series by effectively driving the pumpless systemic venous return directly through the pulmonary circulation. If this arrangement is to work, then two conditions must be fulfilled. First, there must not be any anatomical or functional hindrance along the whole Fontan pathway, from the caval venous inflows to the aortic outflow. Second, there must be a well-suited systemic pump, essentially recruited to the dual function of drawing blood from and, simultaneously, driving blood through, the lungs. This latter function, driving the blood through the lungs, takes place during the venous phase of the systemic output.

More than 30 years ago, Fontan and Baudet proposed bypass of a dysfunctional right ventricle by connecting the pulmonary arteries directly to the right atrium, the so-called atriopulmonary anastomosis. Since then, much experience has been accrued in the field of the functionally univentricular circulation. The proposed connections have been subjected to several modifications, aiming towards minimizing the losses of energy in the cavopulmonary system, and thereby improving the clinical outcomes. A remarkable improvement was achieved with the introduction of the concept of the total cavopulmonary connection, specifically the combination of a bi-directional Glenn anastomosis with a tubular intracardiac extension from the inferior caval venous to the pulmonary arteries. This design was shown to avoid the dissipation of energy associated with the swirling patterns seen in the traditional atrio-pulmonary anastomosis.

Objective:Total extracardiac cavopulmonary connection is an established procedure, but the best spatial arrangement remains controversial. On the basis of our clinical experience with total extracardiac cavopulmonary connection, we performed quantitative and qualitative flow analysis on total extracardiac cavopulmonary connection models simulating the two most frequent arrangements applied to our patients to determine the most favorable hydrodynamic pattern. Methods:We selected two main groups among 110 patients who underwent total extracardiac cavopulmonary connection, those with left-sided inferior vena cava anastomosis (type 1) and those with facing superior and inferior vena cava anastomoses (type 2). Blown-glass total extracardiac cavopulmonary connection phantom models were constructed on the basis of nuclear magnetic resonance and angiographic images. Flow measurements were performed with a Nd:YAG Q-switched laser and a particle imaging velocimetry system. A power dissipation study and a finite-element numeric simulation were also carried out. Results:When applying superior and inferior vena caval flow proportions of total systemic venous return of 40% and 60%, respectively, a vortex was visualized in the type 1 phantom that rotated counterclockwise at the junction of the caval streams. This apparent vortex was not a true vortex; rather, it represented a weakly dissipative recirculating zone modulating the flow distribution into the pulmonary arteries. The power dissipation and finite-element numeric stimulation confirmed the beneficial nature of the apparent vortex and a more energy-saving pattern in the type 1 phantom than in the type 2 phantom. Conclusion:Total extracardiac cavopulmonary connection with left-sided diversion of the inferior vena caval conduit anastomosis is characterized by a central vortex that regulates the caval flow partitioning and provides a more favorable energy-saving pattern than is seen with the total extracardiac cavopulmonary connection with directly opposed cavopulmonary anastomoses.

OBJECTIVES

Our experience with the arterial switch operation for transposition of the great arteries has confirmed the attainability of excellent results with elective neonatal surgery. Up to this time, we have repaired tetralogy of Fallot during the neonatal period only when symptoms, either severe persistent cyanosis or cyanotic spells, have been present This review assesses the results of such nonelective neonatal correction of tetralogy between 1973 and 1988. Twenty-seven neonates with either symptomatic tetralogy of Fallot or symptomatic tetralogy of Fallot with valvar pulmonary atresia underwent repair. Mean age at repair was 8 ± 8.4 days and mean weight was 3.0 ± 0.7 kg. Unsatisfactory palliative shunts had previously been placed elsewhere in four patients. Twenty-five transannular patches and two conduits were used for reconstruction of the right ventricular outflow tract. There were five deaths in the hospital, three of which were due to avoidable technical problems. All deaths occurred in patients with pulmonary artery (Nakata) index less than 150 mm2/m2. One premature child weighing 2.3 kg displayed an absent pulmonary valve-like syndrome after repair and died late of respiratory complications caused by aneurysmal branch pulmonary arteries. Actuarial survival at 5 years was 74%. There was a single rapidly declining hazard phase for death, with the hazard approaching zero at 1½ years after repair. Actuarial freedom from need for reoperation was 76% at 5 years. Postoperative catheterization of 15 long-term survivors showed right ventricular pressure less than 70% systemic in 13 cases. All patients are symptomatically well and functioning in sinus rhythm 1 to 15 years after repair (mean, 5 ± 4 years). This experience with neonates with symptoms suggests that, if mortality is lower in the absence of symptoms, elective repair of tetralogy of Fallot could be reasonably undertaken during the first months of life.

Nineteen patients with truncus arteriosus and single pulmonary artery had corrective operations at the Mayo Clinic from 1969 to 1983. At operation, their ages ranged from 4 months to 20 years (mean 8.1 years). The preoperative pulmonary resistance divided by 2 was used to predict the degree of pulmonary vascular obstructive disease at operation. The influence of elevated pulmonary resistance and the intraoperative postrepair ratio of pulmonary artery to left ventricular pressure on operative and late mortality were examined.

A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare but recognized vascular anomaly that can result in compression of the trachea and the esophagus. This vascular anomaly has been diagnosed using cardiac catheterization and angiography. Recently, computed tomography and magnetic resonance imaging have been used for noninvasive diagnosis. The aim of this report is to highlight the possibility of echocardiographic diagnosis.

The congenital absence of the pulmonary valve cusps can occur either isolated or in association with other heart lesions. We report a very rare case of a 40-day-old infant with transposition of the great arteries, ventricular septal defect, pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the central pulmonary arteries, who received surgical treatment at our institution.