Defining pediatric inpatient cardiology care delivery models: A survey of pediatric cardiology programs in the USA and Canada.

BACKGROUND: The treatment of children with cardiac disease is one of the most prevalent and costly pediatric inpatient conditions. The design of inpatient medical services for children admitted to and discharged from noncritical cardiology care units, however, is undefined. North American Pediatric Cardiology Programs were surveyed to define noncritical cardiac care unit models in current practice. METHOD: An online survey that explored institutional and functional domains for noncritical cardiac care unit was crafted. All questions were multi-choice with comment boxes for further explanation. The survey was distributed by email four times over a 5-month period. RESULTS: Most programs (n = 45, 60%) exist in free-standing children's hospitals. Most programs cohort cardiac patients on noncritical cardiac care units that are restricted to cardiac patients in 39 (54%) programs or restricted to cardiac and other subspecialty patients in 23 (32%) programs. The most common frontline providers are categorical pediatric residents (n = 58, 81%) and nurse practitioners (n = 48, 67%). However, nurse practitioners are autonomous providers in only 21 (29%) programs. Only 33% of programs use a postoperative fast-track protocol. When transitioning care to referring physicians, most programs (n = 53, 72%) use facsimile to deliver pertinent patient information. Twenty-two programs (31%) use email to transition care, and eighteen (25%) programs use verbal communication. CONCLUSION: Most programs exist in free-standing children's hospitals in which the noncritical cardiac care units are in some form restricted to cardiac patients. While nurse practitioners are used on most noncritical cardiac care units, they rarely function as autonomous providers. The majority of programs in this survey do not incorporate any postoperative fast-track protocols in their practice. Given the current era of focused handoffs within hospital systems, relatively few programs utilize verbal handoffs to the referring pediatric cardiologist/pediatrician.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

BACKGROUND: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC). METHODS AND RESULTS: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow-up. Two analyses were performed: (1) propensity-score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site-size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post-S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04). CONCLUSIONS: Among SVD infants in the NPCQIC database discharged post-S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Impact of prenatal diagnosis in survivors of initial palliation of single ventricle heart disease: analysis of the National Pediatric Cardiology Quality Improvement Collaborative database.

Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31-46), and 66% had variants of hypoplastic left heart syndrome. PD was made in 445 (75%), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p < 0.001), there were no differences in birth weight, the presence of major syndromes or other organ system anomalies. Those without PD were more likely to have atrioventricular valve regurgitation (p = .002), ventricular dysfunction (p = 0.06), and pre-operative risk factors including acidosis (p < 0.001), renal insufficiency (p = 0.007), and shock (p = 0.05). Post-operative ventilation was shorter in the PD group (9 vs. 12 d, p = 0.002). Other early post-operative outcomes, interstage course, and outcomes at BDG were similar between groups. In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD showed significant inter-site variation and was associated with improved pre-operative status and shorter duration of mechanical ventilation. The significance of such associations merits further study.

Use of a learning network to improve variation in interstage weight gain after the Norwood operation.

BACKGROUND: Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth. METHODS: A previously identified Nutritional Bundle was spread among NPC-QIC sites. PRIMARY OUTCOME: interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth. RESULTS: Four hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008-December 2010) and 249 in Period 2 (December 2010-April 2013). Median age at S2 was 4.9 months (2.6-12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ <0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was <0 in six sites while in Period 2 no site had median interstage ΔWAZ <0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively). CONCLUSIONS: Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.

Predictors of prolonged length of intensive care unit stay after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

The objective of this study is to identify predictors of prolonged intensive care unit (ICU) length of stay (LOS) for single ventricle patients following Stage I palliation. We hypothesize that peri-operative factors contribute to prolonged ICU stay among children with hypoplastic left heart syndrome (HLHS) and its variants. In 2008, as a part of the Joint Council on Congenital Heart Disease initiative, the National Pediatric Cardiology-Quality Improvement Collaborative established a data registry for patients with HLHS and its variants undergoing staged palliation. Between July 2008 and August 2011, 33 sites across the United States submitted discharge data essential to this analysis. Data describing the patients, their procedures, and their hospital experience were entered. LOS estimates were generated. Prolonged LOS in the ICU was defined as stay greater than or equal to 26 days (i.e., 75th percentile). Statistical analyses were carried out to identify pre-operative, operative, and post-operative predictors of prolonged LOS in the ICU. The number of patients with complete discharge data was 303, and these subjects were included in the analysis. Univariate and multivariate analyses were performed. Multivariate analysis revealed that lower number of enrolled participants (e.g., 1-10) per site, the presence of pre-operative acidosis, increased circulatory arrest time, the occurrence of a central line infection, and the development of respiratory insufficiency requiring re-intubation were associated with prolonged LOS in the ICU. Prolonged LOS in the ICU following Stage I palliation in patients with HLHS and HLHS variant anatomy is associated with site enrollment, circulatory arrest time, pre-operative acidosis, and some post-operative complications, including central line infection and re-intubation. Further study of these associations may reveal strategies for reducing LOS in the ICU following the Norwood and Norwood-variant surgeries.

Prevalence and outcomes of pediatric in-hospital cardiopulmonary resuscitation in the United States: an analysis of the Kids' Inpatient Database*.

OBJECTIVE: Population-based data on pediatric in-hospital cardiopulmonary resuscitation in the United States are scarce. Single-center studies and voluntary registries may skew the estimated prevalence and outcomes. This study aimed to determine the prevalence and outcomes of pediatric cardiopulmonary resuscitation on a national scale. DESIGN: A retrospective analysis of the Healthcare Cost and Utilization Project 2006 Kids' Inpatient Database was performed. Sample weighting was employed to produce national estimates. SETTING: Three thousand seven hundred thirty-nine hospitals in 38 states participating with the Kids' Inpatient Database. PATIENTS: All patients <20 yrs of age hospitalized in participating institutions in 2006. MEASUREMENTS AND MAIN RESULTS: Cardiopulmonary resuscitation was performed in 5,807 (95% confidence interval 5259-6355) children with prevalence of 0.77 per 1,000 admissions. Most patients (68%) were <1 yr old, and 44% were female. On multivariable analysis, cardiopulmonary resuscitation was associated with respiratory failure (odds ratio 41.5, 95% confidence interval 35.4-48.8), myocarditis (odds ratio 36.6, 95% confidence interval 21.9-61.0), acute renal failure (odds ratio 21.6, 95% confidence interval 17.5-26.7), heart failure (odds ratio 3.8, 95% confidence interval 3.0-4.8), and cardiomyopathy (odds ratio 3.8, 95% confidence interval 3.2-4.7). Overall mortality was 51.8% and greater among patients ≥1 yr (68%) vs. <1 yr (44%) (odds ratio 2.7, 95% confidence interval 2.3-3.2). Factors associated with mortality among patients receiving cardiopulmonary resuscitation on multivariable analysis included acute renal failure (odds ratio 1.5, 95% confidence interval 1.1-1.9), hepatic insufficiency (odds ratio 1.5, 95% confidence interval 1.01-2.4), sepsis (odds ratio 1.2, 95% confidence interval 1.01-1.4), and congenital heart disease (odds ratio 1.2, 95% confidence interval 1.01-1.5). CONCLUSIONS: Cardiopulmonary resuscitation is performed in approximately one in 1,300 pediatric hospitalizations. Approximately half of patients receiving cardiopulmonary resuscitation do not survive to discharge. Independent risk factors for mortality after receiving cardiopulmonary resuscitation included congenital heart disease, age ≥1 yr, acute renal failure, hepatic insufficiency, and sepsis.

Variation in preoperative and intraoperative care for first-stage palliation of single-ventricle heart disease: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

BACKGROUND AND METHODS: As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. RESULTS: From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. CONCLUSIONS: In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.

Variation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

BACKGROUND AND METHODS: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. RESULTS: Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common. CONCLUSIONS: Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.

Variation in interstage outpatient care after the Norwood procedure: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

OBJECTIVE: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) is the first quality improvement collaborative in pediatric cardiology, and its registry captures information on interstage care and outcomes of infants after the Norwood procedure. The purpose of this study was to evaluate variation in interstage outpatient clinical care practices for infants discharged home after the Norwood procedure. DESIGN: Data for the first 100 infants enrolled in the NPC-QIC registry were evaluated. The care domains assessed for variation included: (1) discharge communication with outpatient cardiologist and primary care physician (PCP); (2) nutrition plan at hospital discharge; and (3) planned use of home surveillance strategies. RESULTS: One hundred infants were discharged home between July 2008 and February 2010, from 21 participating US pediatric cardiac programs. Median age at discharge was 29 (11-188) days. Interstage outpatient care was provided at the Norwood center for 62 infants, at other centers for 25, and at a combination of centers for 13. Complete discharge communication (defined as written communication of medication list, nutrition plan, and red flag checklist) was relayed to only 45 outpatient cardiologists and to 26 PCPs. Nutrition route at discharge was exclusively oral in 49, combined oral and nasogastric (NG)/nasojejunal (NJ) in 38, exclusively NG/NJ in six, combined oral and gastrostomy tube (GT) in six, and exclusively GT in one infant. Home surveillance strategies were utilized for 81 infants (oximetry and weight monitoring in 77, oximetry alone in four), with no home surveillance in 19 infants. CONCLUSIONS: Considerable variation exists in interstage outpatient care after the Norwood procedure in the care domains of discharge communication, nutrition, and home surveillance. Standardizing care around evidence-based practices may improve the outcomes for these very high-risk children.

Development of a pediatric cardiology quality improvement collaborative: from inception to implementation. From the Joint Council on Congenital Heart Disease Quality Improvement Task Force.

While clinical outcomes in pediatric cardiac disease have improved in recent years, marked institutional and individual cardiology practice variability exists. Quality improvement science has demonstrated that reducing process variation leads to more favorable outcomes, safer practices, cost savings, and improved operating efficiency. This report describes the process undertaken to develop the first collaborative quality improvement project of the Joint Council on Congenital Heart Disease. The project chosen aims to reduce mortality and improve the quality of life of infants with hypoplastic left heart syndrome during the interstage period between discharge from the Norwood procedure and admission for the bidirectional Glenn procedure. The objective of this special article is to inform the pediatric cardiology and cardiac surgery communities of the project to help ensure that the early work by the project pilot participants will spread to clinicians caring for children with cardiovascular disease. It is anticipated that this project will add to our understanding of care for this challenging group of children with hypoplastic left heart syndrome, identifying clinical care changes with the potential to lead to improvements in outcome. It will also introduce the field of pediatric cardiology to the science of collaborative quality improvement and assist in reducing clinical process variation and improving patient outcomes across centers. Finally, it will establish an ongoing network of pediatric cardiologists and their teams linked through a longitudinal data set and collaboration for improvement and research.

Successful transcatheter coil occlusion of a right-sided patent ductus arteriosus with aberrant left subclavian artery.

Right aortic arch is a relatively rare congenital anomaly. The combination of a right aortic arch, a right patent ductus arteriosus, and an aberrant left subclavian artery in a child with an otherwise structurally normal heart is very uncommon. We report the successful transcatheter coil occlusion of a right-sided patent ductus arteriosus in a child with the above-mentioned anatomy. To our knowledge, such a case has not been reported in the English-language medical literature to date.

Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

Spectrum of CHD7 mutations in 110 individuals with CHARGE syndrome and genotype-phenotype correlation.

CHARGE syndrome is a well-established multiple-malformation syndrome with distinctive consensus diagnostic criteria. Characteristic associated anomalies include ocular coloboma, choanal atresia, cranial nerve defects, distinctive external and inner ear abnormalities, hearing loss, cardiovascular malformations, urogenital anomalies, and growth retardation. Recently, mutations of the chromodomain helicase DNA-binding protein gene CHD7 were reported to be a major cause of CHARGE syndrome. We sequenced the CHD7 gene in 110 individuals who had received the clinical diagnosis of CHARGE syndrome, and we detected mutations in 64 (58%). Mutations were distributed throughout the coding exons and conserved splice sites of CHD7. Of the 64 mutations, 47 (73%) predicted premature truncation of the protein. These included nonsense and frameshift mutations, which most likely lead to haploinsufficiency. Phenotypically, the mutation-positive group was more likely to exhibit cardiovascular malformations (54 of 59 in the mutation-positive group vs. 30 of 42 in the mutation-negative group; P=.014), coloboma of the eye (55 of 62 in the mutation-positive group vs. 30 of 43 in the mutation-negative group; P=.022), and facial asymmetry, often caused by seventh cranial nerve abnormalities (36 of 56 in the mutation-positive group vs. 13 of 39 in the mutation-negative group; P=.004). Mouse embryo whole-mount and section in situ hybridization showed the expression of Chd7 in the outflow tract of the heart, optic vesicle, facio-acoustic preganglion complex, brain, olfactory pit, and mandibular component of the first branchial arch. Microarray gene-expression analysis showed a signature pattern of gene-expression differences that distinguished the individuals with CHARGE syndrome with CHD7 mutation from the controls. We conclude that cardiovascular malformations, coloboma, and facial asymmetry are common findings in CHARGE syndrome caused by CHD7 mutation.

Atrial septal stent implant: atrial septal defect creation in the management of complex congenital heart defects in infants.

OBJECTIVES: Certain congenital heart defects require the creation of an unrestrictive atrial septal defect (ASD) to relieve atrial hypertension, to maintain systemic cardiac output, or to achieve adequate atrial mixing to improve systemic oxygen saturation. We describe a series of patients, ranging in age from 5 weeks to 17 months, in whom we implanted a stent across the atrial septum to create a lasting, unrestrictive interatrial communication. METHODS: Five patients with left heart obstructive defects presented with severe left atrial hypertension. The patients weighed 3.5-10.1 kg. Patients 1 and 2 presented at 5 and 9 weeks of age, respectively. Patient 1 had a restrictive patent foramen ovale, and patient 2 had an intact atrial septum. Patients 3, 4, and 5 had previous cardiac surgery including atrial septectomy. At presentation, all patients were poor surgical candidates. RESULTS: Using standard percutaneous technique, a premounted Palmaz-Genesis stent (Cordis Corporation) was implanted across the atrial septum in all 5 patients, using balloons ranging from 7 to 10 mm diameter. Each patient had a dramatic decrease in left atrial pressure and increased ASD diameter. All stented ASDs remained widely patent, confirmed by Doppler echocardiography, until elective surgical stent explant. CONCLUSION: Patients presenting beyond the neonatal period with left heart obstructive defects and intact atrial septum or restrictive ASD present a challenging problem. We report a safe and effective technique for relief of left atrial hypertension in infants using premounted intravascular stents. All patients had immediate marked hemodynamic improvement.

Genetic predictors and remodeling of dilated cardiomyopathy in muscular dystrophy.

BACKGROUND: Dystrophin gene mutations cause 2 common muscular dystrophies, Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). Both are frequently associated with dilated cardiomyopathy (DCM) and premature death. We hypothesized that early diagnosis and treatment of DCM in DMD/BMD patients would lead to ventricular remodeling and that specific dystrophin gene mutations would predict cardiac involvement. METHODS AND RESULTS: Sixty-nine boys with DMD (n=62) and BMD (n=7) (mean age, 12.9 and 13.7 years, respectively) were referred to our Cardiovascular Genetics Clinic for evaluation, including echocardiography and DNA analysis. Follow-up evaluations were scheduled yearly until the first abnormal echocardiogram indicative of DCM and quarterly thereafter. After the first abnormal echocardiogram, angiotensin-converting enzyme inhibitor or beta-blocker therapy was started. beta-Blockers were added if echocardiography showed no ventricular remodeling in angiotensin-converting enzyme inhibitor-treated patients after 3 months. DCM was diagnosed in 31 subjects (DMD, 27/62, 44%; BMD, 4/7, 57%) (mean age at onset, 15.4+/-2.8 years; range, 10.4 to 21.2 years). All 31 subjects were begun on pharmacological therapy after diagnosis. On follow-up (n=29), 2 subjects (both DMD) showed stable DCM, 8 subjects (all DMD) showed improvement, and 19 subjects (16 DMD; 3 BMD) showed normalization of left ventricular size and function (total improvement, 27/29 [93%]). DNA analysis in 47 cases (68%) revealed a significant association between DCM and exon 12 and 14 to 17 mutations, possible protection against DCM by exon 51 to 52 mutations, and a trend toward significant association between onset of DCM and exon 31 to 42 mutations. Statistical significance was based on nominal probability values. CONCLUSIONS: Early diagnosis and treatment of DCM may lead to ventricular remodeling in DMD/BMD patients. Specific dystrophin gene mutations appear to be predictive of cardiac involvement, while other mutations may protect against or inhibit development of DCM. Further studies evaluating the impact of early intervention strategies on left ventricular geometry and function in muscular dystrophy patients seem warranted.

Use of cutting balloon for palliative treatment in tetralogy of Fallot.

In tetralogy of Fallot, severe cyanosis due to insufficient pulmonary blood flow necessitates early intervention. The Cutting Balloon, developed for percutaneous coronary angioplasty, was used successfully for transcatheter incision and dilation of infundibular and valvar pulmonary stenosis in four patients with tetralogy of Fallot.

Sudden death in congenital heart disease.

Sudden cardiac death is a common mechanism of demise in association with congenital cardiac abnormalities. The varied mechanisms may include failure of the transitional circulation, arrhythmias, postoperative or perioperative complications in the neonate and coronary ischaemia, arrhythmias, sepsis, thrombosis, or pulmonary hypertensive crisis in the older child. Knowledge of the natural history of unoperated and operated forms of congenital heart disease and long term follow up with the detection and treatment of underlying hemodynamic abnormalities should improve outcomes. There are few patients with congenital cardiac anomalies that are cured and most require long term care.

Is cardiac catheterization a prerequisite in all patients undergoing bidirectional cavopulmonary anastomosis?

Traditionally, all patients undergo cardiac catheterization before bidirectional cavopulmonary anastomosis (BCPA). The purpose of this study was to determine if preoperative catheterization is necessary when echocardiographic parameters appear favorable. A retrospective review was performed of all patients who underwent BCPA (n = 142) between February 1996 and May 2001. Echocardiographic criteria defining a favorable BCPA candidate included good ventricular function, moderate or less atrioventricular and semilunar valve regurgitation, absence of ventricular outflow tract obstruction, normal proximal branch pulmonary artery (PA) size, and low PA pressures estimated by PA band gradient or systemic PA shunt velocity. The median age at operation was 7 months (range: 2-11) and weight was 6.2 kg (range: 2.7-7.1). There were 73 unfavorable candidates. Patients with hypoplastic left heart syndrome (n = 23) and pulmonary atresia with intact septum (n = 15) predominated among the unfavorable group. All patients were catheterized. This provided additional information on PA pressures in 3 patients in the favorable group but did not defer operation or influence outcome (no mortality, prolonged pleural drainage, or longer intensive care department stay). All 3 patients are alive at a mean follow-up of 51 months. The 30-day mortality was 2% (4 of 151 patients), all in the unfavorable group. Overall, 20 patients (13%) required arterioplasty of PAs at the time of BCPA. BCPA can be performed with a low risk of morbidity and mortality in a wide range of patients. By using commonly acquired echocardiographic parameters, a low-risk subgroup of patients can be identified who can safely avoid preoperative cardiac catheterization.