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Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients Olaf Reinhartz, MD, V

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Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients Olaf Reinhartz, MD, V CARDIOVASCULAR Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients Olaf Reinhartz, MD, V. Mohan Reddy, MD, Edwin Petrossian, MD, Sam Suleman, BS, Richard D. Mainwaring, MD, David N. Rosenthal, MD, Jeffrey A. Feinstein, MD, Raj Gulati, MD, and Frank L. Hanley, MD Department of Cardiothoracic Surgery, Division of Pediatric Cardiac Surgery, and Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Stanford, California Background. Unifocalization of major aortopulmonary procedures. Median postoperative pulmonary artery collateral arteries (MAPCAs) in pulmonary atresia with pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg ventricular septal defect and intracardiac repair has be- (Fontan), respectively. Six patients are alive today (46%), come the standard of care. However, there are no reports with 1 patient lost to follow-up. Three patients died early addressing unifocalization of MAPCAs in single-ventri- and 3 late after initial unifocalization to shunts. One cle patients. It is unknown whether their pulmonary other patient survived unifocalization, but was not con- vascular bed can be reconstructed and low enough pul- sidered a candidate for a Glenn procedure and died after monary vascular resistance achieved to allow for superior high-risk two-ventricle repair. Another patient with or total cavopulmonary connections. right-ventricle–dependent coronary circulation died of Methods. We reviewed data on all patients with func- sepsis late after Glenn. tional single ventricles and unifocalization procedures of Conclusions. In selected patients with functional single MAPCAs. From 1997 to 2005, 14 consecutive children ventricles and MAPCAs, the pulmonary vascular bed can with various single-ventricle anatomies were operated be reconstructed sufficiently to allow for cavopulmonary on. connections. Venous flow to the pulmonary vasculature Results. Patients had a median of three surgical proce- decreases cardiac volume load and is likely to increase dures (range, 1 to 5). Two patients had absent, all others life expectancy and quality of life for these patients. diminutive central pulmonary arteries, with an average (of 3.5 ؎ 1.2 MAPCAs. Seven patients (50%) had bidirec- (Ann Thorac Surg 2006;82:934–9 tional Glenn procedures, and 3 of these had Fontan © 2006 by The Society of Thoracic Surgeons he surgical treatment of pulmonary atresia with ven- Even though the vast majority of patients with T tricular septal defect (VSD) and major aortopulmo- MAPCAs express the intracardiac anatomy of tetralogy of nary collaterals (MAPCAs) has evolved substantially over Fallot with pulmonary atresia, there are some rare excep- the last 2 decades [1–7]. In the early 1990s, our group tions. There are case reports in the literature of MAPCAs started a protocol of early unifocalization of MAPCAs in association with pulmonary atresia with intact septum and intracardiac repair in these patients. We follow these (PA/IVS) [12, 13] and with right isomerism [14, 15].We general principles: (1) early single-stage complete unifo- have also seen MAPCAs with a variety of other defects calization of all sources of pulmonary blood flow and with biventricular and univentricular morphology. Sin- intracardiac repair whenever feasible [8, 9]; (2) complete gle-ventricle patients account for 4.1% of the patients unifocalization to a shunt if intracardiac repair can not undergoing unifocalizations in our experience. This yet be accomplished; (3) creation of an aortopulmonary study focuses on the surgical treatment of patients with (AP) window to the true pulmonary arteries (PAs) in MAPCAs and single-ventricle physiology, including both appropriate cases of very small PAs with normal ar- true single-ventricle morphology such as PA/IVS, tricus- borization [10]; and (4) staged unifocalization through pid atresia, or severely unbalanced common atrioventric- thoracotomies in unfavorable cases of severe segmental ular canal (CAVC), and complicated two-ventricle mor- level stenoses of MAPCAs. This protocol has been suc- cessful in achieving a high rate of early complete repair phology that is deemed unseptatable. We describe our with low right ventricular pressures [11]. experience with this challenging group of patients. Accepted for publication March 20, 2006. Patients and Methods Presented at the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30–Feb 1, 2006. All patients with MAPCAs and single-ventricle physiol- ogy referred to our surgical group at Stanford University Address correspondence to Dr Reinhartz, Stanford University, Depart- ment of Cardiothoracic Surgery, 300 Pasteur Dr, Stanford, CA 94305; or one of our associated hospitals were retrospectively e-mail: [email protected]. reviewed. Current follow-up was obtained by facsimile © 2006 by The Society of Thoracic Surgeons 0003-4975/06/$32.00 Published by Elsevier Inc doi:10.1016/j.athoracsur.2006.03.063 Ann Thorac Surg REINHARTZ ET AL 935 2006;82:934–9 UNIFOCALIZATION IN SINGLE-VENTRICLE PATIENTS Fig 1. Preoperative angiograms of a 3-month-old infant with com- plex, nonseptatable L-transposition and pulmonary atresia who under- CARDIOVASCULAR went aortopulmonary window, right thoracotomy unifocalization of major aortopulmonary collateral arteries, and bidirectional Glenn shunt. (A) Multiple aortopulmo- nary collateral arteries arising from different levels of the de- scending aorta. (B) Diminutive true pulmonary arteries connecting to a right upper lobe collateral. from the patients’ pediatric cardiologists. The study was rectional Glenn shunts for volume unloading as early as approved by the Administrative Panel on Human Sub- possible, and we completed total cavopulmonary connec- jects at Stanford University on January 5, 2006. Individual tion (Fontan circulation) whenever appropriate. Both patient consent was waived owing to the retrospective these procedures were preferably done avoiding cardio- nature of the study. pulmonary bypass, using passive shunts intraopera- tively. Concomitant abnormalities such as total anoma- Pulmonary Blood Supply lous pulmonary venous return were corrected early as MAPCAs were defined as major collateral arteries arising well. from the arterial circulation in the setting of pulmonary atresia. Typically, these vessels arise from the proximal Statistics descending aorta; however, frequently also from su- Owing to the small number of patients, no complex praaortic branches or other aortic segments. True pulmo- statistical analyses were performed. nary arteries were either absent or diminutive and sup- plied only by MAPCAs. Patients with discontinuous Results pulmonary arteries such as those due to closure of a persistent ductus arteriosus or iatrogenically (that did not Between 1997 and 2005, 14 consecutive patients with have true MAPCAs) were excluded, as well as patients MAPCAs and single ventricles (6 male, 8 female) pre- with acquired aortopulmonary collaterals only, such as sented to our institution. Median age at first operation they frequently occur in cyanotic heart disease. was 3 months (range, 5 days to 9 years). Eight of these patients were referred from other states. Intracardiac Morphology Only patients with pulmonary atresia were included. Morphology and Additional Morbidities Patients had either true single-ventricle cardiac morphol- Patients had an average of 3.5 Ϯ 1.2 MAPCAs. The ogy or two-ventricle hearts that could not be surgically average number of MAPCAs was similar in survivors (3.3 septated and therefore had to be palliated like single Ϯ 1.0) and nonsurvivors (3.7 Ϯ 1.5). Two patients had ventricles. Patients with forms of tetralogy or balanced completely absent intrapericardial PAs; 1 of these 2 had a double-outlet right ventricle (DORV) as well as all other Fontan connection and survived, the other died late after septatable hearts were excluded. In fact, the majority of a Glenn procedure. All other patients had diminutive patients referred to us with MAPCAs and complex biven- PAs. Figure 1 shows a preoperative angiogram of a tricular morphology such as corrected transposition had 3-months old infant with multiple collaterals (Fig 1A) and unifocalizations along with intracardiac repairs and are diminutive true PAs (Fig 1B). consequently not included in this study. There was a variety of intracardiac morphologies, which are listed in Table 1, along with concomitant Surgical Protocol cardiac defects. A number of patients had significant In general, we applied similar treatment principles to this associated noncardiac morbidities: Six patients had patient population as to those with pulmonary atresia/ asplenia, 4 had dextrocardia, and 1 velocardiofacial syn- VSD as mentioned in the introduction section. All pa- drome. Two patients had severe tracheobronchomalacia, tients were completely unifocalized to shunts as early as 1 of them requiring tracheostomy postoperatively. possible. In selected cases, AP windows or staged thora- cotomy unifocalizations were performed initially. Surgical Procedures In addition, we applied other generally accepted con- A total of 40 surgical procedures were performed in these cepts of single-ventricle palliation. We performed bidi- patients. The median number of operations per patient CARDIOVASCULAR 936 REINHARTZ ET AL Ann Thorac Surg UNIFOCALIZATION IN SINGLE-VENTRICLE PATIENTS 2006;82:934–9 Table 1. Spectrum of Intracardiac Morphology and Early Deaths Concomitant Cardiac Defects Three patients died early after unifocalization proce- Number dures
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