Long-term progression and survival following Norwood Single Ventricle Reconstruction.

PURPOSE OF REVIEW: Much data exist concerning Norwood discharge mortality. Less is known about late survival. Examining the available data in light of the Single Ventricle Reconstruction trial is insightful as focus shifts toward long-term survival. RECENT FINDINGS: Data from 2000 to 2001 demonstrated approximately 40-50% 10-year survival, 30-40% or less between 10 and 15 years. The shape of the curves was characteristic; the majority of deaths within the first year, followed by a late constant phase. Publications from 2001 to 2005 suggested that various combinations of technical and perioperative modifications allowed hospital discharge survivals as high as 90-94%. As results matured (2005-2010) a consistent message was that, although the shape of the newer curves was similar (highest hazard in the first 1 year), higher hospital survival shifted the later phase to yield better long-term survival (70-85% between 5 and 10 years). Some emphasized right ventricle-based shunts as a 'cause' of improving results. Since 2010, the Single Ventricle Reconstruction trial has matured and has increasingly shifted opinion away from the right ventricle shunt as a 'cause' of improved results. The survival of the right ventricle shunt group is slightly higher at 3 years, but the 1-year statistical significance has been lost and the two groups converge. As the Single Ventricle Reconstruction study was based on the interaction between randomized shunt and survival, the secondary and other endpoint analyses must be cautiously considered. SUMMARY: The current English-language literature suggests a 60-80% 5-10 year survival expectation. The shape of the survival curve remains; the highest hazard remains the first year before a later, stable phase is reached. Rather than a 'magic bullet' theory surrounding one technique or practice, centers have differentially adopted various combinations to optimize Norwood survival. Optimizing interstage I survival is a challenge to further increase the percentage of patients reaching the late, stable phase.

Chondrosarcoma presenting with pulmonary embolism in a 9-year-old girl: a case report.

Chondrosarcoma is a malignant bone tumour common in adults, third to myeloma and osteosarcoma, but is exceptionally rare in children. Here we discuss a 9-year-old girl presenting with occlusive right pulmonary artery neoplastic embolus, resulting from a primary right proximal humerus chondrosarcoma. To the best of our knowledge, this the first pediatric and only second overall case reported in the United States of a neoplastic pulmonary embolus resulting from a primary chondrosarcoma.

Off-pump snare technique for congenital left atrial appendage aneurysm.

Left atrial appendage aneurysm is an extremely rare anomaly and as such has been rarely imaged or seen intraoperatively with very little accumulated management experience. The available scant published literature stresses resection on cardiopulmonary bypass as the safest and by far the most commonly applied technique. We suggest a novel alternative imaging-guided management utilising an off-pump tourniquet snare technique under live transoesophageal echocardiography.

Transient bilateral blindness in a patient after cardiac surgery.

Our report describes a case of temporary bilateral blindness in a patient after undergoing mitral valve replacement with a mechanical prosthesis and coronary artery bypass grafting.

Combined lung and liver transplantation: the United States experience.

BACKGROUND: Combined transplantation of the lungs and liver is indicated for patients who would not be expected to survive transplantation of either organ alone. No single center has accumulated a significant experience, and as a result the expectations for this operation in the current era are unknown. METHODS: Patients that have undergone combined lung-liver transplantation in the United States were enrolled through the United Network for Organ Sharing Organ Procurement and Transplantation Network database. In addition, the English-language literature was searched for additional cases of combined lung-liver transplantation. RESULTS: Eleven patients have undergone combined lung and liver transplantation in the United States at different centers. The 1- and 5-year patient survival rates are of 79% and 63%, respectively, and no patient has required retransplantation. These patient survival rates are equivalent to similar a combined lung-liver case series from the United Kingdom (P=0.37, log-rank test) and isolated orthotopic liver transplantation in the United States (P=0.59, log-rank test), and are comparable to patient survival rates following isolated lung transplantation in the United States. CONCLUSIONS: Patient survival of combined lung-liver transplantation is comparable to that of isolated liver and isolated bilateral lung transplantation. This option should be considered for patients with end-stage lung disease and liver disease when transplantation of a single organ transplantation is precluded by severe disease in the other organ system.

Lessons learned from the first application of the DeBakey VAD Child: an intracorporeal ventricular assist device for children.

We report and describe the design and the first clinical implantation of the DeBakey ventricular assist device (VAD) Child, a pediatric intracorporeal left ventricular assist device, in a 6-year-old girl. The risk-benefit of novel technologies in advanced heart failure and the lessons learned by our experience are important to consider in hopes that other centers (where this device is now available) may benefit.

Intermediate Results of Hybrid Versus Primary Norwood Operation.

BACKGROUND: In 2007 we began a hybrid program for hypoplastic left heart syndrome (HLHS) variants to potentially improve outcome in high-risk patients. During implementation we offered both hybrid and Norwood approaches to all risk categories. The purpose of this study was to perform a comparative analysis of intermediate survival. METHODS: Newborns were evaluated jointly for high-risk characteristics, including birth weight less than 2.5 kg, prematurity (especially < 35 weeks), central nervous system abnormalities, multiorgan failure, intact or severely restrictive atrial septum, severe ventricular dysfunction, and severe atrioventricular valve regurgitation. We prefer Norwood for standard risk and hybrid for high risk, but all groups crossed over into all treatment pathways resulting in the following 5 treatment groups: standard risk Norwood; high-risk Norwood; standard risk hybrid ductal stent (HDS); high-risk hybrid DS; and high-risk hybrid prostaglandin E1 (HPGE). We reviewed all consecutive patients from 2007 to 2012, obtained follow-up, and analyzed the results. RESULTS: Sixty-eight newborns presented (median 2.96 kg, 8 days); 29 (43%) were high and 39 (57%) were standard risk. There were 14 stage I hospital deaths strongly associated with risk: 3 of 39 standard (7.7%) and 11 of 29 high (38%, p = 0.002). Stage I discharge mortality was highest for high-risk Norwood and high-risk HPGE groups (p < 0.001). Actuarial survival up to 5 years demonstrated superior survival for Norwood versus hybrid (78.1% vs 56.4%, p = 0.0182). With risk stratification there was suboptimal survival for all 3 high-risk groups (p = 0.003); HDS fared better than HPGE but had higher birth weight (p < 0.001). CONCLUSIONS: While a risk-stratified approach for HLHS variant patients with selective use of hybrid palliation resulted in acceptable stage I mortality, the longer term mortality for high-risk patients remains higher than for standard risk regardless of treatment modality. Intrinsic patient risk factors (rather than treatment modality) likely determine long-term outcome in experienced centers. Our current high-risk approach has evolved to HPGE application with Norwood conversion whenever deemed medically possible.

Recurrent neoaortic insufficiency after the switch back operation with previous repair of transposition with ventricular septal defect and aortic arch hypoplasia.

Neoaortic insufficiency is not uncommon after the arterial switch operation (ASO) for d-Transposition, yet surgery is rarely required. In a patient with worsening neoaortic regurgitation post-arterial switch and ventricular septal defect (VSD) closure, we performed a successful "switch back" operation with documented aortic valve competence on discharge echocardiography. However, recurrent severe aortic insufficiency required valve replacement, and histopathology of the excised valve indicated abnormal leaflet/vascular wall structure. We question whether the switch back operation is a viable option for neoaortic insufficiency after an ASO in patients with previous d-Transposition and VSD, when the native pulmonary valve may have structural deficiencies.

Left ventricular inflow obstruction associated with persistent left superior vena cava and dilated coronary sinus.

BACKGROUND: It has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition. METHODS: Since 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial "a" waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients. RESULTS: There were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient. CONCLUSIONS: We conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique.

Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch.

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.

Proximal aortic reoperations in patients with composite valve grafts.

BACKGROUND: The purpose of this study was to examine our experience with proximal aortic reoperations in patients with composite valve grafts (CVGs) and assess postoperative survival and morbidity. METHODS: Since 1991, 33 patients with CVGs underwent reoperation for one or more of the following indications: aneurysms distal to the CVG (n = 20, 61%), false aneurysms (n = 13, 39%) and graft infection (n = 7, 21%). Operations included false aneurysm repair (n = 13, 39%), graft replacement of distal ascending aortic or transverse aortic arch aneurysm (n = 20, 61%) and aortic root re-replacement with a new CVG (n = 6, 18%) or homograft (n = 4, 12%). RESULTS: Operative mortality was 15% (n = 5), including 2 of the 7 patients who had infected CVGs (29%). All 4 patients who had infected CVGs replaced with aortic root homografts survived. Complications included vocal cord paralysis (n = 4, 12%), bleeding requiring reoperation (n = 3, 9%) and stroke (n = 2, 6%). Actuarial 3-year survival was 74.4% +/- 7.9%. CONCLUSIONS: Reoperations in patients with CVGs remain challenging procedures with high associated morbidity and mortality, especially in the setting of graft infection. The results of homograft aortic root re-replacement for infected CVGs are encouraging.

Acute respiratory failure after pleurodesis with doxycycline.

Bedside pleurodesis through a tube thoracostomy has been shown to be effective treatment of malignant pleural effusion and pneumothorax with persistent air leak. A variety of agents can be used, and each has been shown to produce rare but potentially serious complications. We report a case of sudden, severe respiratory failure in a 42-year-old man after pleurodesis with 300 mg of doxycycline. His response was consistent with an anaphylactic reaction. After intubation, mechanical ventilation and nebulizer treatments, he rapidly recovered to baseline. On the basis of this report and a review of the literature, we believe that doxycycline may not be an innocuous agent for bedside pleurodesis and that such procedures warrant a monitored setting.

Current readings: long-term management of patients undergoing successful pediatric cardiac surgery.

As of 2000, more adults than children are alive with congenital heart disease. Each year, more of these adults with congenital heart disease undergo surgery. Adults with congenital heart disease require lifelong surveillance, follow-up imaging, and clinical decision making by appropriately trained and familiar physicians and extenders. Three common challenges facing adults with congenital heart disease are the neglected patient, weak programmatic infrastructure, and the planning and management of pregnancy in the adult with congenital heart disease. Many challenges must be overcome in order to establish and maintain a comprehensive medical and surgical program for adults with congenital heart disease. Three common and challenging groups of adults with congenital heart disease who may require surgery are adults requiring tricuspid valve surgery for Ebstein's malformation, those requiring reoperation for failing Fontan circulation, and those requiring pulmonary valve replacement (PVR). Surgery for Ebstein's anomaly is now offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the Maze procedure), and coronary artery bypass grafting. Patients undergoing previous iterations of the Fontan operation are especially prone to arrhythmia and failing circulation. Fontan conversion with arrhythmia surgery and pacemaker therapy was developed by Constantine Mavroudis, Barbara Deal, and Carl Backer to treat these challenging patients, with the first such operation performed in 1994. Fontan conversion involves: (1) conversion of the previously created atriopulmonary connection to a total cavopulmonary connection (TCPC), extracardiac Fontan; (2) arrhythmia surgery, typically with a modified biatrial Maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and (3) concomitant procedures to treat associated lesions. The need for pulmonary valve replacement is increasing for many adults with congenital heart disease. In the past, chronic pulmonary regurgitation following repair of tetralogy of Fallot was considered benign. Current evidence demonstrates that chronic pulmonary regurgitation causes significant morbidity by producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for pulmonary valve replacement including several recent developments such as pulmonary valve replacement with a hand-sewn polytetrafluoroethylene bicuspid valve and percutaneous pulmonary valve replacement. Reoperative cardiac surgery is common in adults with congenital heart disease. Although a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, patients with the greatest number of previous surgeries appear to be a higher risk group. Multi-institutional data about adults with congenital heart disease from The Society of Thoracic Surgeons Congenital Heart Surgery Database can be used to estimate prognosis and council patients and their families. The six manuscripts reviewed in this article have been selected to give a flavor of the state of the art in the domain of caring for adults with congenital heart disease and to provide important information about the long term management of patients undergoing successful pediatric cardiac surgery.

Anomalous aortic origin of the left coronary artery: in-hospital cardiac arrest and death despite bed rest.

A ten-year-old female was admitted with syncope and a myocardial infarction, was resuscitated, and was diagnosed with anomalous left coronary artery from right aortic sinus. After initial stabilization, she was on bed rest in the intensive care unit awaiting surgery and experienced sudden arrest and could not be resuscitated, resulting in death.

Osteosarcoma with cardiac metastasis in a 22-year-old man: a case report and review of cardiac tumors.

Primary osteosarcoma accounts for 3% of all childhood cancer. It commonly occurs during the adolescent growth spurt and is more common in boys than girls and in African Americans than white people. The 5-year survival is approximately 79%. Cardiac metastasis of osteosarcoma is exceedingly rare; we present an unusual case in a 22-year-old man with significant intracardiac tumor burden. Additionally, we review the current pediatric cardiac tumor literature.

Porcine xenograft aortic root replacement in a three month old with severe truncal insufficiency.

Outcomes for truncus arteriosus repair are impacted significantly by the severity of truncal valve dysfunction. When satisfactory repair of the regurgitant truncal valve is unattainable, replacement is required. Given our experience in children with stentless porcine xenografts in the aortic position and the incidence of early valve failure for aortic homografts in infants, we replaced a severely regurgitant truncal valve with a full-root porcine xenograft in a 3-month-old infant. The initial and early result are encouraging, suggesting that the stentless porcine xenograft may be considered an option in cases where primary repair of the truncal (or aortic) valve is not possible.

Left ventricular diverticulum: how we do it.

Left ventricular diverticulum is an extremely rare anomaly, especially in the absence of other findings, and as such it has been rarely imaged, rarely seen intraoperatively, and has no standardized management algorithm. We report our suggested imaging and management in the context of previous management philosophies.