Phillip S. Naimo

Outcomes of Truncus Arteriosus Repair in Children: 35 Years of Experience From a Single Institution

We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection.

Outcomes of aortopulmonary window repair in children: 33 years of experience.

BACKGROUND The purpose of this study was to assess the outcomes of children undergoing repair of aortopulmonary window (APW). METHODS We conducted a retrospective review of all children (n=43) who underwent surgical repair of APW between 1980 and 2013. RESULTS Median age at surgery was 40 days (range, 13 to 125). Simple APW was present in 15 of 43 patients (35%), and 28 of 43 patients (65%) patients had concomitant cardiovascular anomalies. The aorta was repaired by direct suturing in 36 patients (84%) patients and patching in 7 patients (16%). The main pulmonary artery was repaired by direct suturing in 22 patients (51%) patients and by patching in 21 (49%). Cardiopulmonary bypass was used in 42 of the 43 patients (97.7%). Single-staged repair of concomitant cardiovascular anomalies was undertaken in 26 of 28 patients (93%). Only 2 of the 28 patients (7%) underwent repair of interrupted aortic arch before APW repair. Operative mortality was 6.7% (1 of 15 patients) among patients with simple APW and 18% (5 of 28 patients) among patients with concomitant anomalies. Operative weight less than 2.5 kg was associated with mortality on univariable analysis (p=0.02). Median follow-up was 10.1 years (range, 0.17 to 24.2). There were no late deaths. Overall survival was 86% (95% confidence interval: 71.3 to 94.2) at 10 years. Freedom from reoperation was 95.3% (95% confidence interval: 86.2 to 99.9) at 10 years. At last follow-up, all patients were in New York Heart Association functional class I/II. CONCLUSIONS Survival beyond discharge from the hospital is associated with excellent outcomes.

Double-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch

A persistent fifth aortic arch is exceptionally rare. We report a patient with a double-lumen aortic arch in association with a perimembranous ventricular septal defect and Cornelia de Lange syndrome. We also discuss the morphologic consequences of persistent fifth aortic, arch, which may not be limited to the double-lumen aorta but in fact may be more common.

Anomalous Aortic Origin of the Left Coronary Artery From the Right Coronary Sinus: Diagnosis and Surgical Repair of Intramural Retrovalvular Coronary Artery.

Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct translocation of the left coronary artery could be difficult due to its intramural course and proximity to the aortic valve. We described the surgical management of a child after sudden hemodynamic collapse and the coronary translocation technique that prevented distortion of the aortic root and allowed successful translocation of the abnormal coronary artery despite its intramural course immediately behind the aortic valve commissure.

Long-term outcomes of complete vascular ring division in children: a 36-year experience from a single institution.

Objectives Complete vascular rings are rare and cause tracheoesophageal compression. Following surgical division, some patients have persisting tracheomalacia. We aim to assess the long-term outcomes of complete vascular ring division. Methods All patients (n = 132) who underwent surgical division of a complete vascular ring between 1978 and 2014 were identified from the hospital database and retrospectively reviewed. Results Complete vascular rings consisted of a double aortic arch (n = 80), right aortic arch with an aberrant subclavian artery and left ligamentum arteriosum (n = 50), right aortic arch with mirror image branching and left ligamentum arteriosum (n = 1), and a left aortic arch with right descending aorta and right ligamentum arteriosum (n = 1). Kommerells diverticulum was identified in 10 patients. Preoperative tracheomalacia was identified via bronchoscopy in 25 patients. Concomitant tracheal reconstruction was not performed in any patient. Kommerells diverticulum was resected in 1 patient. The hospital mortality rate was 1.5% (2/132). There were no late deaths. The overall survival rate was 98.3 ± 1.2% (95% CI: 93.4, 99.6) at 20 years. Postoperatively, persistent tracheal compression was reported in 3 patients, and tracheomalacia in 16 patients. The rate of freedom from reoperation was 88.6 ± 4.0% (95% CI: 77.9, 94.3) at 20 years. No patient required tracheal surgery during the follow-up period. Follow-up was 92% (121/132) complete, with a median follow-up of 11.4 years (range 44 days to 36 years). At the last follow-up, 7 patients had mild tracheomalacia. Conclusions Outcomes of division of a complete vascular ring are excellent. Tracheomalacia often improves following division of the vascular ring. Respiratory symptoms following complete vascular ring division are uncommon.

Prevention of Right Pulmonary Artery Stenosis in Fontan Circulation: The Melbourne Modification of T-Fontan Operation

Currently, the extracardiac Fontan operation is a procedure of choice in patients undergoing a staged palliation for univentricular hearts. However, it is not always easy to prevent the right pulmonary artery twisting after implantation of the extracardiac conduit. Herein, we described a simple modification, which we referred to as T-Fontan procedure, to prevent right pulmonary artery stenosis after extracardiac Fontan operation.

Aspirin resistance in the era of personalized medicine: Should we not take it personally?

An interesting and thought-provoking article by DeNino and colleagues 1 appears in the current issue of the Journal. This article describes 2 patients with HeartMate II (Thoratec Corporation, Pleasanton, Calif) left ventricular assist device (LVAD) in whom pump thrombosis developed despite what appeared to be an appropriate initial anticoagulation regimen. Both initially received aspirin at low dose (81 mg daily), heparin, and warfarin. The initial responsiveness to aspirin, defined as less than 550 aspirin reaction units (ARU), was confirmed in both patients. Both became unresponsive to aspirin. Both appeared noncompliant with their anticoagulation regimens. Did both have aspirin resistance, or resistance to taking aspirin? How can one determine the causes of diverse prothrombotic conditions that are pulled together and called ‘‘aspirin resistance’’? What isthis elusiveaspirinresistance anyway? The article by DeNino and colleagues 1 poses more questions than it gives answers. Yet one has to begin somewhere, and this article is an excellent beginning. It does not provide the solution, but rather it brings attention to a complex problem yet to be solved. As we enter the era of personalized medicine, a few comments on aspirin resistance and its relevance to cardiac surgeons appear timely.Aspirin resistance is a real phenomenon and defined as the inability of aspirin to reduce platelet production of thromboxane A2 (TXA2; Figure 1) and thereby platelet activation and aggregation. 2 Aspirin is a nonselective and irreversible inhibitor of both forms of cyclooxygenase (COX), but is weakly more selective for COX-1. Aspirin inhibition of COX-1 is rapid and saturable at low doses. This may leave some COX-2, which is greatly upregulated by proinflammatory conditions, uninhibited. Thus TXA2 can be produced by activated macrophages, monocytes, and endothelial cells through COX-2. Interestingly, higher doses of aspirin cause greater inhibition of COX-2 and dramatically lower concentration of urinary 11-dehydrothromboxane B2. 3,4 The Heart Outcomes Prevention Evaluation study 5 demonstrated that patients

Outcomes of repair of left partial anomalous pulmonary venous connection in children

Herein, we report a case series of patients who underwent repair of left partial anomalous pulmonary venous connection (L-PAPVC) via anastomosing the anomalous pulmonary vein (PV) to the left atrial appendage. Fifteen children underwent repair of L-PAPVC between 1980 and 2014. The median age at surgery was 3.6 years (range: 5 days to 17.2 years). Concomitant anomalies were present in 87% (13/15). There were no early deaths. There was 1 late death occurring 63 days following surgical repair due to pneumococcal septicaemia in a patient with prior atrial septal defect closure and Ehlers-Danlos syndrome. The overall survival rate was 93.7% at 15 years. A single patient (1/15, 7%) required reoperation 1 year after L-PAPVC repair for PV stenosis due to several thrombi located throughout the PV. The rate of freedom from PV reoperation was 90% at 10 years. The follow-up was 100% complete with a median time of 11 years (range: 52 days to 20 years). To our knowledge, this is the youngest cohort of patients who have undergone surgical repair of L-PAPVC. Repair of L-PAPVC in children can be achieved via anastomosis of the anomalous vessel to the left atrial (LA) with excellent outcomes. The rate of anastomotic stenosis at the site of implantation on the LA is low.

Impact of truncal valve surgery on the outcomes of the truncus arteriosus repair

OBJECTIVES Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution. METHODS We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180 and severe: 1.1%, 2/180). RESULTS Twenty-one patients had concomitant TV surgery with an early mortality of 19% (4/21) and overall survival of 70.8 ± 10.1% at 25 years. There were 60 neonates, 11 of whom had concomitant TV surgery with an early mortality of 27% (3/11) and overall survival of 62.3 ± 15.0% at 20 years. Concomitant TV repair (P = 0.5) was not a risk factor for death. TV reoperation was common in those who had concomitant TV surgery, with freedom from reoperation of 19.2 ± 14.9% at 20 years. In the remaining 159 patients, 14 required subsequent TV surgery, and the freedom from TV surgery was 84.0 ± 4.6% at 20 years. At a median follow-up of 18.5 years, TV insufficiency was none or trivial in 79.6% (109/137) and mild or less in 98.5% (135/137) of patients. CONCLUSIONS Most patients with mild TV insufficiency are free from TV surgery up to 25 years. The durability of TV repair is poor. Most patients with moderate or greater TV insufficiency and a quadricuspid TV will require TV surgery.

Small Incisions for Small Children: Is Right Lateral Thoracotomy a Right Approach in Open Heart Surgery in Infants?

An interesting article by Li and colleagues (1) is published in the current issue of Heart, Lung and Circulation that compares cosmetic right lateral thoracotomy (89 patients) to the conventional midline sternotomy approach for ventricular septal defect (VSD) closure (116 patients) in infants weighing less than 5 kg. In this retrospective study, both groups of infants were operated in Beijing Anzhen Hospital during the same time period and the choice of incision depended on the preference of the surgeon. The authors concluded that infants with right thoracotomy had shorter operative time, shorter length of incision, less drainage and transfusion. A few comments seem to be appropriate to put this excellent article into proper prospective. Currently, repair of simple intracardiac anomalies is a very low risk surgery and children after such surgery lead normal life. Thus, anything less than a perfect outcome after such surgery is not acceptable. Therefore, the enthusiasm in cosmetic approach has traditionally been somewhat tempered. Nonetheless, a cosmetically superior incision that does not compromise patient’s safety is clearly important. Thus, cosmetic incision in the axillary area is very appealing in selected patients. The evidence accumulated during the last 15 years suggests that right lateral thoracotomy in children does not increase mortality and morbidity [1–9] (Table 1). Li and colleagues (1) for the first time demonstrated safety and efficacy of right lateral thoracotomy in small infants undergoing VSD closure. While