Search

Anomalous origin of the left coronary artery from the pulmonary artery, also known as Garland-Bland-White syndrome, usually occurs as an isolated condition. We report an infant with caudal regression sequence diagnosed with interrupted aortic arch type B and type III aortopulmonary window, who was found to have anomalous origin of the left coronary artery from the right pulmonary artery at surgical repair. Successful repair of the aortopulmonary window and interruption was performed with reimplantation of the left coronary artery into the ascending aorta. This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair.

Background . BioGlue surgical adhesive (CryoLife, Inc, Kennesaw, GA) is currently being used to secure hemostasis at cardiovascular anastomoses in adults. Interference with vessel growth would preclude its use during congenital heart surgery. The purpose of this study was to determine if BioGlue reinforcement of aortic anastomoses impairs vessel growth and causes strictures. Methods . Ten 4-week-old piglets (8.0 ± 1.4 kg) underwent primary aorto-aortic anastomoses. Five piglets were randomly assigned to anastomotic reinforcement with BioGlue. After a 7-week growth period, the aortas were excised for morphometric analysis and histopathology. Results . Weight gains were similar in both groups. In BioGlue animals, however, aortic circumference increased only 1.5 ± 0.8 mm (versus 2.7 ± 0.8 mm in controls; p = 0.054). BioGlue animals developed a 33.9% stenosis of the aortic lumen area (versus 3.7% in controls, p = 0.038). Adventitial changes reflecting tissue injury and fibrosis were present in all BioGlue animals versus none of the control animals ( p = 0.008). Conclusions . BioGlue reinforcement impairs vascular growth and causes stricture when applied circumferentially around an aorto-aortic anastomosis. This adhesive should not be used on cardiovascular anastomoses in pediatric patients.

Objective. We sought to document the prevalence of Internet access and usage patterns among families who have children with congenital heart disease presenting for cardiac surgery. Design. A cross-sectional study was performed utilizing a questionnaire (10 questions) as the study tool. Statistical analysis was performed on all completed questionnaires. The χ2 test was used to evaluate categorical variables and the t test to evaluate continuous variables. Results. Two hundred seventy-five questionnaires were completed and analyzed. Thirty-seven percent (102/275) of the children had a cyanotic congenital heart defect. There were 21 children with Trisomy 21. Fifty-eight percent (160/275) of families had access to the Internet. The most common locations for accessing the Internet were home (80%; 129/160) and work (51%; 82/160). There were no significant differences in Internet access with regards to underlying individual congenital heart defect, cyanotic versus acyanotic heart defects, or congenital heart defects with functional univentricular hearts versus biventriuclar hearts. Families with older children (12–24 years) were more likely to have Internet access. Families of children undergoing placement of a right ventricle to pulmonary artery conduit were more likely to have Internet access. Of the 160 families with Internet access, 58% (93/160) used the Internet to obtain information related to their child’s cardiac diagnosis. Eighty-two percent (76/93) characterized locating cardiology-related information as easy. Six parents created interactive personal Web sites specifically related to their child’s congenital heart defect. Although families with older patients (12–24 years) were more likely to have access to the Internet, this did not translate into greater use of the Internet to obtain cardiology-related information. Among families who accessed the Internet for cardiology-related information, 95% (88/93) of families characterized the information as helpful or very helpful in furthering the understanding of their child’s heart defect. Conclusion. Families are utilizing the Internet to educate themselves about congenital heart disease. Most parents consider the process easy and the information obtained helpful to the understanding of their child’s congenital heart defect and surgery. Internet use in this patient population is expected to increase. Our vigilance in providing accurate Internet references, as well as in identifying inaccurate Internet information available to our patients and their parents, is of paramount importance.

Pediatric myocardial related morbidity and mortality after cardiopulmonary bypass (CPB) are well documented, but the effects of pulsatile perfusion (PP) versus nonpulsatile perfusion (NPP) on myocardial blood flow during and after hypothermic CPB are unclear. After investigating the effects of PP versus NPP on myocardial flow during and after hypothermic CPB, we quantified PP and NPP pressure and flow waveforms in terms of the energy equivalent pressure (EEP) for direct comparison. Ten piglets underwent PP (n = 5) or NPP (n = 5). After initiation of CPB, all animals underwent 15 minutes of core cooling (25 degrees C), 60 minutes of hypothermic CPB with aortic cross-clamping, 10 minutes of cold reperfusion, and 30 minutes of rewarming. During CPB, the mean arterial pressure (MAP) and pump flow rates were 40 mm Hg and 150 ml/kg per min, respectively. Regional flows were measured with radiolabeled microspheres. During normothermic CPB, left ventricular flow was higher in the PP than the NPP group (202+/-25 vs. 122+/-20 ml/l 00 g per min). During hypothermic CPB, no significant intragroup differences were observed. After 60 minutes of ischemia and after rewarming (276+/-48 vs. 140+/-12 ml/100 g per min; p < 0.05) and after CPB (271+/-10 vs. 130+/-14 ml/100 g per min; p < 0.05), left ventricular flow was higher in the PP group. Right ventricular flow resembled left ventricular flow. The pressure increase (from MAP to EEP) was 10+/-2% with PP and 1% with NPP (p < 0.0001). The increase in extracorporeal circuit pressure (ECCP) (from ECCP to EEP) was 33+/-10% with PP and 3% with NPP (p < 0.0001). Pulsatile flow generates significantly higher energy, enhancing myocardial flow during and after hypothermic CPB and after 60 minutes of ischemia in this model.

Objective Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA. Methods This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded. Results Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n = 2) or a systemic-to-PA shunt (n = 6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA. Conclusions PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy.

An 11-month-old girl was transferred for consideration of cardiac transplantation. She had previously undergone repair of complete atrioventricular septal defect at another institution. Her postoperative course was notable for severe mitral regurgitation, pulmonary hypertension, and heart failure. At reoperation, the left atrioventricular valve was considered irreparable with a very small (11 mm) annulus. Using a technique to enlarge the mitral annulus, a 17-mm prosthetic valve was placed. Her postoperative course was unremarkable and she is doing very well at 3 years follow-up.

UNLABELLED We compared the ventilation and pulmonary mechanics produced by a new anesthesia ventilator (NAD 6000) using a circle system with that produced by a critical care ventilator (Servo 900C) using a nonrebreathing circuit in infants with congenital heart disease. Twenty patients, aged 1 day to 7 mo, weighing 2.1 to 4.6 kg, were studied. The NAD 6000 had improved alveolar ventilation: PaCO(2) 43 +/- 8 vs 47 +/- 5 mm Hg (P = 0.005), end-tidal CO(2) 34 +/- 7 vs 37 +/- 5 mm Hg (P = 0.042); larger inspired tidal volumes 12.9 +/- 2.8 vs 11.3 +/- 2.2 mL/kg (P < 0.001), but with higher mean airway pressures 9.7 +/- 1.6 vs 8.6 +/- 1.3 cm H(2)O (P < 0.001). These differences in ventilation and airway pressures were not clinically significant. Although there were differences in observed ventilatory variables, both machines provided adequate ventilation when set in the volume control mode. IMPLICATIONS We compared two ventilators for use in infants. Twenty infants undergoing surgery for congenital heart defects were randomized to receive ventilation first with one ventilator, then with the other. Although there were differences in observed ventilatory variables, both machines provided adequate ventilation when set in the volume control mode.

We investigated the effects of two new hollow-fiber membrane oxygenators, the Capiox SX10 and the Lilliput 901, on pulsatile versus nonpulsatile perfusion in an in vitro model designed to simulate a 3 kg infant. The experiments were divided into eight groups (six pulsatile and two nonpulsatile), according to the equipment and settings used. Each group included six tests. In all experiments, the pseudo-patient’s mean arterial pressure was 40 mmHg, and the pump flow rate was 550 ml/min. During pulsatile cardiopulmonary bypass, the pump’s base flow was set at 30%, and the pump rate was set at 80, 100, 120, 140, or 150 beats/min. The PUMP START and PUMP STOP timing points were adjusted to produce different pulse-width settings. We were especially interested in evaluating the pre- and postoxygenator extracorporeal circuit pressure (ECP), the oxygenator pressure drop, and the precannula ECP. When used with a pulsatile roller pump, the Capiox produced a significantly lower preoxygenator ECP than the Lilliput ( p < 0.001); moreover, the Capiox yielded a significantly lower oxygenator pressure drop ( p < 0.001). During nonpulsatile perfusion, the Capiox again produced a lower preoxygenator ECP than the Lilliput ( p < 0.001). These results suggest that the Capiox may be more suitable than the Lilliput when the pulsatile flow is employed, and pulsatile flow does not increase the ECP with either oxygenator.

An alternate physiologic pulsatile pump (PPP) system was designed and evaluated to produce sufficient pulsatility during neonate-infant open heart surgery. This hydraulically driven pump system has a unique "dual" pumping chamber mechanism. The first chamber is placed between the venous reservoir and oxygenator and the second chamber between the oxygenator and patient. Each chamber has two unidirectional tricuspid valves. Stroke volume (0.2-10 ml), upstroke rise time (10-350 msec), and pump rate (2-250 beats per minute [bpm]) can be adjusted independently to produce adequate pulsatility. This system has been tested in 3-kg piglets (n = 6), with a pump flow of 150 ml/kg/min, a pump rate of 150 bpm, and a pump ejection time of 110 msec. After initiation of cardiopulmonary bypass (CPB), all animals were subjected to 25 minutes of hypothermia to reduce the rectal temperatures to 18 degrees C, 60 minutes of deep hypothermic circulatory arrest (DHCA), then 10 minutes of cold perfusion with a full pump flow, and 40 minutes of rewarming. During CPB, mean arterial pressures were kept at less than 50 mm Hg. Mean extracorporeal circuit pressure (ECCP), the pressure drop of a 10 French aortic cannula, and the pulse pressure were 67+/-9, 21+/-6, and 16+/-2 mm Hg, respectively. All values are represented as mean+/-SD. No regurgitation or abnormal hemolysis has been detected during these experiments. The oxygenator had no damping effect on the quality of the pulsatility because of the dual chamber pumping mechanism. The ECCP was also significantly lower than any other known pulsatile system. We conclude that this system, with a 10 French aortic cannula and arterial filter, produces adequate pulsatility in 3 kg piglets.

A 3-month-old patient with heterotaxy syndrome, complex intracardiac malformations, and severe heart failure underwent surgical correction. Anatomy included an interrupted inferior vena cava with hemiazygous continuation to a persistent left superior vena cava draining to the left atrium. The presence of partial anomalous pulmonary venous drainage precluded an intraatrial baffle. Systemic venous reconstruction was achieved using the left atrial appendage along with an intracardiac repair of the other defects.

Purpose Ventricular assist device (VAD) support in heart transplant recipients is challenging especially because these patients are immunocompromised. Total artificial heart (TAH) is an attractive alternative to VAD for adult patients since it alleviates the need for immunosuppression. Unlike for adults, TAH is not currently available for children due to size limitations. The aim of this study is to determine if VAD support, currently the only realistic option, plays a role in the treatment of cardiac graft failure in the pediatric population. Methods and Materials A retrospective analysis was performed of all pediatric heart transplant (Tx) recipients who required VAD support at Texas Children’s Hospital for cardiac graft dysfunction. We identified 16 VAD runs in 15 patients; 9 with short-term extracorporeal VAD and 7 with long-term VAD. The type of device was chosen based on the etiology of heart failure; short-term VAD for acute process (e.g. acute rejection) and long-term VAD for chronic process (e.g. transplant coronary artery disease). Median age and weight at VAD placement were 11y (1–18y) and 43kg (9–94kg). Median time from Tx to VAD support was 1.1y (0d–2.7y) for short-term VADs and 3.6y (10m–11y) for long-term VADs. Results Short-term VAD support was successfully weaned off following cardiac recovery in 8 (89%), with support duration of 5d (2-7d). One patient was bridged to long-term VAD. In contrast, successful outcome was achieved only in 2 (29%) patients with long-term VAD support, both of whom were bridged to re-transplant with support duration of 28 and 83 days. The remaining 5 (71%) died on long-term VAD. None of the 2 patients who required resuscitation with ECMO prior to long-term VAD survived. Of note, 3 of the 5 mortalities were related to fungus infection. Conclusions Short-term VAD support is an effective therapy for acute graft failure. Long-term VAD support, however, has a limited role for chronic graft failure. The search for alternative treatment strategies for children with chronically failing grafts is warranted.

Purpose Thoracic multiorgan transplantations (TMOTx) are complex procedures that when performed in adults have shown similar survival to single organ transplantation (TX). The overall number of TMOTx has been gradually increasing, however, the absolute number remains low compared to single organ TX. In the pediatric population, TMOTx constitute only a small fraction of the total pediatric TXs performed. A national database was used to review the overall experience with pediatric TMOTx in the United States. Methods and Materials United Network of Organ Sharing database was queried through 2011 for pediatric (≤18y) TMOTx. Of the 2102 TMOTx, 238 (12%) were pediatric TMOTx: 181 (76%) were heart-lung TX, while others included heart-kidney (32,13%), lung-liver (11,5%), heart-liver (10,4%), heart-lung-liver (3,1%) and lung-kidney (1,1%). TMOTx accounted for 3% (238/7945) of the total pediatric thoracic TX performed. Results Analysis of outcomes shows that graft survival in combined heart-lung TX was similar to survival in lung-only TX (p=0.127) but was worse than in heart-only TX (p Figure 1 ). Similarly, graft survival for TMOTx involving lung, except lung-liver (p=0.05), was not different than lung-only survival ( Figure 2 ). Conclusions Thoracic multiorgan transplantation offers a promising option for pediatric patients with multiorgan failure, while combining abdominal organ transplant with heart or lung transplant does not portend a worse outcome. Although rare in the pediatric population, these procedures can be performed successfully with an expectation of similar graft survival to single organ transplantation.

The Use of Short-Term Ventricular Assist Devices in Children I. Adachi, J.S. Heinle, E.D. McKenzie, M.C. Mcgarry, A.G. Cabrera, A. Jeewa, C.D. Fraser, Jr., D.L.S. Morales. Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Baylor College of Medicine, Houston, TX; PediatricsCardiology, Baylor College of Medicine, Houston, TX; Cardiovascular Surgery, Texas Children’s Hospital, Houston, TX.

[1] Antunes PE, Anacleto G, Ferrao de Oliveira JM, Eugenio L, Antunes MJ. Staged carotid and coronary surgery for concomitant carotid and coronary artery disease. Eur J Cardiothorac Surg 2002;21:181–186. [2] Minami K, Fukahara K, Boethig D, Bairaktaris A, Fritzsche D, Koerfer R. Long-term results of simultaneous carotid endarterectomy and myocardial revascularization with cardiopulmonary bypass used for both procedures. J Thorac Cardiovasc Surg 2000;119:764–773.

We report the case of a 6-year old girl who presented with severe dilated cardiomyopathy. Cross-sectional echocardiography demonstrated a severely dilated hypokinetic left ventricle with an ejection fraction less than 10%. She developed intractable ventricular tachyarrhythmias and subsequently had a left ventricular assist device implanted. Within 1 week of mechanical support, echocardiography demonstrated dramatic reverse remodeling of the left ventricle with marked improvement in myocardial deformation using strain rate imaging. This report further highlights the potential for rapid remodeling after mechanical support of the failing myocardium.

Underlying mechanisms in the development of atrial flutter or intra-atrial re-entry tachycardia in patients with structural cardiac abnormalities remain poorly defined. The right atrial myocardium from two patients with congenital heart disease was evaluated, of whom one presented with severe right atrial dilation and arrhythmia and the other with a normal right atrium, to assess whether increased right atrial pressure and volume overload give rise to sarcolemmal alteration. N-terminus dystrophin staining in the atrium from the patient who had undergone a Fontan procedure showed a normally distributed but significantly reduced staining signal compared with the second patient. This is the first report that patients with severe right atrial dilation and atrial flutter have marked reduction in atrial dystrophin expression.

An 11-month-old female underwent cardiology evaluation during hospital admission for apnea. Physical examination revealed 2 distal pulses in the upper extremity and slightly diminished pulses in the lower extremity. Noninvasive blood pressures were as follows: right arm 107/76, left arm 91/51, right leg 107/55, left leg 94/76. Echocardiogram revealed left aortic arch, but coarctation of the aorta could not be ruled out and cardiac catheterization was performed. Catheterization revealed the presence of a persistent left fifth aortic arch (Fig 1). Ascending aortic pressure was 105/49. Because access to the femoral arteries could not be obtained and the catheter could not be passed beyond the isthmus, noninvasive cuff pressure of the left lower extremity was measured concomitantly and was 85/45, giving a systolic gradient of 20 mm Hg. Double left aortic arch (DLAA) as a result of persistent left fifth aortic arch is a very rare premortem diagnosis. Postmortem cardiac pathologic collection has suggested that the incidence may be higher, largely due to failure of recognition. In 1999, there had been 26 reported cases of DLAA and ten of these (38%) were associated with coarctation of the aorta [1]. Of these, five (50%) were associated with interruption of the left fourth arch. Persistent left fifth aortic arch can exist in both systemic-tosystemic configuration (such as this case) and in systemic-to-pulmonary configuration. The latter is extraordinarily difficult to differentiate from patent ductus arteriosis. The presence of double left aortic arch is not, in itself, an indication for intervention.