Inferior Outcomes on the Waiting List in Low-Volume Pediatric Heart Transplant Centers.

Low case volume has been associated with poor outcomes in a wide spectrum of procedures. Our objective was to study the association of low case volume and worse outcomes in pediatric heart transplant centers, taking the novel approach of including waitlist outcomes in the analysis. We studied a cohort of 6482 candidates listed in the Organ Procurement and Transplantation Network for pediatric heart transplantation between 2002 and 2014; 4665 (72%) of the candidates underwent transplantation. Candidates were divided into groups according to the average annual transplantation volume of the listing center during the study period: more than 10, six to 10, three to five, or fewer than three transplantations. We used multivariate Cox regression analysis to identify independent risk factors for waitlist and posttransplantation mortality. Of the 6482 candidates, 24% were listed in low-volume centers (fewer than three annual transplantations). Of these listed candidates in low-volume centers, only 36% received a transplant versus 89% in high-volume centers (more than 10 annual transplantations) (p < 0.001). Listing at a low-volume center was the most significant risk factor for waitlist death (hazard ratio [HR] 4.5, 95% confidence interval [CI] 3.5-5.7 in multivariate Cox regression and HR 5.6, CI 4.4-7.3 in multivariate competing risk regression) and was significant for posttransplantation death (HR 1.27, 95% CI 1.0-1.6 in multivariate Cox regression). During the study period, one-fourth of pediatric transplant candidates were listed in low-volume transplant centers. These children had a limited transplantation rate and a much greater risk of dying while on the waitlist.

Clostridium difficile colitis in children following lung transplantation.

Risk factors for Clostridium difficile diarrhea are antibiotic exposure, hospitalization, extreme ages, and immunodeficiency. Patients with CF have a high rate of colonization with C. difficile. We performed a retrospective chart review of patients at Texas Children's Hospital who underwent lung transplantation since the inception of our program in October 2002 until October 2008. There were 78 pediatric lung transplants performed at our institution during the study period. Four patients developed six total episodes of CDC for an overall incidence of 5.4%. CF was the underlying diagnosis in all four patients, leading to an incidence of 8.9% in patients with CF. Two patients developed colitis within the first four months following transplant, and the other two patients developed colitis more than three yr after transplantation. All four patients required hospitalization, and three patients were managed medically while one patient underwent diverting ileostomy. One experienced renal insufficiency and subsequently expired. Overall survival was 75% among patients with CDC following lung transplantation. CDC causes significant morbidity and mortality in children with CF who have undergone lung transplantation.

Mycobacterium abscessus in cystic fibrosis lung transplant recipients: report of 2 cases and risk for recurrence.

Mycobacterium abscessus is increasingly recognized as an important pathogen in some individuals with advancing lung disease related to cystic fibrosis (CF). Because of its resistance to antimicrobial agents and virulence, its presence in the lungs of potential lung transplant recipients can be problematic. We present 2 cases of individuals with CF in whom M. abscessus was present in the preoperative sputum cultures. The organism manifested different degrees of invasiveness in the 2 cases after transplantation with different outcomes, suggesting an approach to future candidates for lung transplantation that may be of clinical significance to their physicians and surgeons.

Lung transplantation in a patient with a thrombophilic disorder.

The prothrombin G20210A mutation has been associated with an increased risk of graft failure in renal transplant recipients. Little is known about the potential effect of this mutation on lung transplant recipients. We report the case of bilateral lung transplantation in a patient with cystic fibrosis who was heterozygous for the G20210A mutation of the prothrombin gene.

Persistence of bilateral arterial ducts in pulmonary atresia despite confluent branch pulmonary arteries: opportunity for two percutaneous therapeutic alternatives.

We present a case of a newborn infant with double inlet left ventricle, pulmonary atresia, confluent pulmonary arteries, and bilateral arterial ducts (AD), to discuss the therapeutic alternatives offered by interventional catheterization techniques in this anatomic arrangement. The infant initially underwent stenting of the right AD to stabilize pulmonary blood flow off of prostaglandin infusion. Three weeks later, she developed left pulmonary artery isolation upon closure of the left arterial duct. An additional stent was placed in the pulmonary artery confluence, restoring blood flow to the left lung and significantly improving her oxygen saturations. At 6 months of age she underwent her first surgical procedure, a successful bidirectional cavopulmonary anastomosis with removal of the left pulmonary artery stent and patch enlargement of the pulmonary artery confluence. She continues to do well in clinical follow-up at 16 months of age.

Pulmonary venous stenosis as an associated manifestation of left-sided obstructive lesions in an infant with partial atrioventricular canal defect.

Patients with partial atrioventricular canal defect (PAVC) who present with congestive heart failure within the first year of life are usually found to have associated left-sided obstructive lesions. We present a case of a child with PAVC who subsequently developed progressive pulmonary venous stenosis as a manifestation of her disease.

Anesthetic and perioperative outcome of teenagers and adults with congenital heart disease.

OBJECTIVE: To compare the perioperative outcome of patients >or=13 years old undergoing surgery for congenital heart disease in a children's hospital by a dedicated congenital heart surgery and anesthesia team with procedure-matched younger control patients. DESIGN: Retrospective medical record review study. From October 1997 to July 2000, medical records of all patients >12 years old requiring cardiopulmonary bypass were reviewed. A control group of patients

Perioperative care of the adult with congenital heart disease in a free-standing tertiary pediatric facility.

Adult patients with congenital heart disease are presenting more frequently for cardiac surgery. Frequently, pediatric congenital heart surgeons perform these procedures at pediatric hospitals. Between July 1995 and June 2000, a retrospective review of adult patients (> or = 18 years old) who had undergone cardiothoracic operations was performed. A total of 112 operations were performed and divided into two groups--81 cardiac operations in 79 patients and 31 noncardiac operations in 23 patients. One patient had a cardiac and noncardiac operation performed. The overall early operative mortality was 6% (6/101). There were 3 late deaths. New-onset cardiac arrhythmias requiring treatment were diagnosed after 5/81 (6%) cardiac operations. Six of 79 (7%) patients were diagnosed with postoperative clinical depression. An acceptable mortality can be achieved when adult patients undergo cardiothoracic operations at a pediatric facility. New-onset arrhythmias necessitating treatment are relatively common, and postoperative clinical depression should be anticipated.

Tetralogy of Fallot: surgical management individualized to the patient.

BACKGROUND: Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity. METHODS: Based on approximately 40 years' experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock-Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months. RESULTS: From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction. CONCLUSIONS: This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.

Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanyl-midazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics.

BACKGROUND: The cardiovascular effects of halogenated anesthetic agents in children with normal hearts have been studied, but data in children with cardiac disease are limited. This study compared the effects of halothane, isoflurane, sevoflurane, and fentanyl-midazolam on systemic and pulmonary hemodynamics and myocardial contractility in patients with congenital heart disease. METHODS: Fifty-four patients younger than age 14 scheduled to undergo congenital heart surgery were randomized to receive halothane, sevoflurane, isoflurane, or fentanyl-midazolam. Cardiovascular and echocardiographic data were recorded at baseline and at randomly ordered 1 and 1.5 minimum alveolar concentrations, or predicted equivalent fentanyl-midazolam plasma concentrations. The shortening fraction and ejection fraction (using the modified Simpson rule) were calculated. Cardiac index was assessed by the velocity-time integral method. RESULTS: Halothane caused a significant decrease in mean arterial pressure, ejection fraction, and cardiac index, preserving only heart rate at baseline levels. Fentanyl-midazolam in combination caused a significant decrease in cardiac index secondary to a decrease in heart rate; contractility was maintained. Sevoflurane maintained cardiac index and heart rate and had less profound hypotensive and negative inotropic effects than halothane. Isoflurane preserved both cardiac index and ejection fraction, had less suppression of mean arterial pressure than halothane, and increased heart rate. CONCLUSIONS: Isoflurane and sevoflurane preserved cardiac index, and isoflurane and fentanyl-midazolam preserved myocardial contractility at baseline levels in this group of patients with congenital heart disease. Halothane depressed cardiac index and myocardial contractility.

Volume ventilation of infants with congenital heart disease: a comparison of Dräger, NAD 6000 and Siemens, Servo 900C ventilators.

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.

Intraoperative diagnosis of aortic pseudoaneurysm with transesophageal echocardiography.

A pseudoaneurysm of the ascending aorta is a rare complication of aortic valve endocarditis that requires prompt diagnosis. Several imaging strategies can be used; however, transesophageal echocardiography (TEE) has been utilized more frequently due to its superior resolution in detection of aortic valve complications. This case presents a patient with prosthetic valve dysfunction in which intraoperative TEE was used to diagnose a previously undetected aortic pseudoaneurysm, thus leading to a change in surgical management.

Cardiac herniation producing tamponade: the critical role of early diagnosis.

BACKGROUND: Rupture of the pleuropericardium (PP) occurs rarely, with most patients dying of associated injuries before arriving at the hospital. Among patients who initially survive, the diagnosis is often delayed until cardiogenic shock secondary to cardiac herniation is evident. METHODS: The records of 10 patients with PP lacerations and cardiac herniations were reviewed. RESULTS: All but one patient had a normal chest x-ray (CXR) film on admission. After the patients became symptomatic, seven of nine had abnormal findings on CXR film demonstrating herniation of the heart into the left hemithorax. The other two patients underwent surgery without a repeat CXR film. Except for one who was taken directly to the operating room, all patients had been previously stabilized before developing cardiogenic shock, on average 9 hours after admission. Operative therapy was closure of the pericardium for five patients and completion pericardiotomy for the others. All survivors developed significant complications, and four of them died. CONCLUSIONS: The diagnosis of PP rupture should be considered for patients with multiple trauma who develop sudden and unexpected cardiogenic shock after their initial condition has been stabilized. A repeat CXR film is diagnostic in most cases and should be used as the most efficient and expeditious route to making the diagnosis.