Univentricular Congenital Heart Disease Research Articles

Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design.

AimsIn univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV‐INHIBITION study rationale, design, and methods.Methods and resultsThe SV‐INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo‐controlled, Phase III study, aiming to evaluate the efficacy of sildenafil on the ventilatory efficiency during exercise, in teenagers and adult patients (>15 years old) with an SV. Patients with a mean pulmonary arterial pressure >15 mmHg and a trans‐pulmonary gradient >5 mmHg, measured by cardiac catheterization, will be eligible. The primary outcome is the variation of the VE/VCO2 slope, measured by a cardiopulmonary exercise test, between baseline and 6 months of treatment. A total of 50 patients are required to observe a decrease of 5 ± 5 points in the VE/VCO2 slope, with a power of 90% and an alpha risk of 5%. The secondary outcomes are clinical outcomes, oxygen saturation, 6 min walk test, SV function, NT‐proBNP, peak VO2, stroke volume, mean pulmonary arterial pressure, trans‐pulmonary gradient, SF36 quality of life score, safety, and acceptability.ConclusionsThe SV‐INHIBITION study aims to answer the question whether PDE5 inhibitors should be prescribed in patients with an SV. This trial has been built focusing on the three levels of research defined by the World Health Organization: disability (exercise tolerance), deficit (SV function), and handicap (quality of life).

TREATMENT OF SUBACUTE FONTAN FAILURE USING A CATHETER-BASED APPROACH TO FONTAN TAKEDOWN

Following Fontan palliation for univentricular congenital heart disease (uCHD), life-threatening complications related to this unique physiology often develop. Surgical takedown (TD) is associated with a 40% mortality, and few options exist for refractory Fontan failure. A 5-year-old with

Hepatic Vein Blood Increases Lung Microvascular Angiogenesis and Endothelial Cell Survival—Toward an Understanding of Univentricular Circulation

To improve our understanding of pulmonary arteriovenous malformations in univentricular congenital heart disease, our objective was to identify the effects of hepatic vein and superior vena cava constituents on lung microvascular endothelial cells independent of blood flow. Paired blood samples were collected from the hepatic vein and superior vena cava in children 0-10 years old undergoing cardiac catheterization. Isolated serum was subsequently used for in vitro endothelial cell assays. Angiogenic activity was assessed using tube formation and scratch migration. Endothelial cell survival was assessed using proliferation (BrdU incorporation, cell cycle analysis) and apoptosis (caspase 3/7 activity, Annexin-V labeling). Data were analyzed using Wilcoxon signed-rank test and repeated measures analysis. Upon incubating lung microvascular endothelial cells with 10% patient serum, hepatic vein serum increases angiogenic activity (tube formation, P = 0.04, n = 24; migration, P< 0.001, n = 18), increases proliferation (BrdU, P < 0.001, n = 32; S-phase, P = 0.04, n = 13), and decreases apoptosis (caspase 3/7, P < 0.001, n = 32; Annexin-V, P = 0.04, n = 12) compared to superior vena cava serum. Hepatic vein serum regulates lung microvascular endothelial cells by increasing angiogenesis and survival in vitro. Loss of hepatic vein serum signaling in the lung microvasculature may promote maladaptive lung microvascular remodeling and pulmonary arteriovenous malformations.

Overview of adult congenital heart transplants.

Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, the indications and potential contraindications for transplant, surgical considerations, and post-transplant outcomes. The findings reveal that 3.3% of adult heart transplants occur in ACHD patients. The potential mechanisms for the development of late ventricular dysfunction include a morphologic systemic right ventricle, altered coronary perfusion, and ventricular noncompaction. The indications for transplant in ACHD patients include systemic ventricular failure refractory medical therapy, Fontan patients failing from chronic passive pulmonary circulation, and progressive cyanosis leading to functional decline. Transplantation in ACHD patients can be quite complex and may require extensive reconstruction of the branch pulmonary arteries, systemic veins, or the aorta. Vasoplegia, bleeding, and graft right ventricular dysfunction can complicate the immediate post-transplant period. The post-transplant operative mortality ranges between 14% and 39%. The majority of early mortality occurs in ACHD patients with univentricular congenital heart disease. However, there has been improvement in operative survival in more contemporary studies. In conclusion, the experience with cardiac transplantation for ACHD patients with end-stage heart failure is growing, and high-risk patient subsets have been defined. Significant strides have been made in developing evidence-based guidelines of indications for transplant, and the intraoperative management of complex reconstruction has evolved. With proper patient selection, more aggressive use of mechanical circulatory support, and earlier referral of patients with failing Fontan physiology, outcomes should continue to improve.

Anaesthetic Management of Parturients With Univentricular Congenital Heart Disease and the Fontan Operation

(Int J Obstet Anesth. 2016;28:83–91) Over the past few decades, as surgical treatment of congenital heart disease (CHD) has improved, an increasing number of women with CHD are becoming pregnant and obstetric anesthesiologists must be able to manage these women during labor and delivery. One of the newest challenges is that presented by women with a single ventricular circulation that has been palliated with the Fontan operation.

Modified Aortic Uncrossing Procedure: A Novel Approach for Norwood Palliation of Complex Univentricular Congenital Heart Disease With a Circumflex Aorta

The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual. We report such a case and describe our approach to its surgical management.

Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease

Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair. In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years). Our analysis categorized participants in these three groups: with structurally normal hearts as controls (n=21), with BiV circulation (n=20) and with UniV circulation (n=14). We acquired ESE images of the systemic ventricle including pulsed-wave flow and spectral tissue Doppler imaging (TDI) of lateral free wall before and immediately after standard, symptom-limited exercise tests on an electronically braked cycle ergometer. During ESE we obtained inflow E-wave and TDI systolic (S') and early diastolic (E') velocities in 93% to 100% of participants at rest and in 90% to 100% of participants post exercise. Feasibility to obtain Doppler imaging parameter was the same across study groups. The myocardial response to exercise was increase in heart rate (HR), S' and inflow E-wave velocity in all participants. Patients with BiV circulation had preserved ventricular function at rest. While patients with UniV circulation had low S', E', and E-wave velocities at rest in comparison to controls and to BiV group (all P<.001), both patients with BiV and UniV circulation showed significant increases in HR, S' velocity and inflow E-wave velocity post exercise, with magnitudes of these increases higher in controls than in the BiV and UniV group. The S' and E' velocities were strongly associated with lower percent predicted peak oxygen consumption VO2 (rs=0.614 and rs=0.64, respectively, both P<.001). ESE with Doppler imaging is a practical noninvasive diagnostic method and sufficiently robust for the assessment of morphologic LV/systemic ventricles under exercise in patients after biventricular and univentricular CHD repair. Although patients with BiV and UniV circulation had both preserved myocardial response to exercise, the magnitude of this response was the lowest in patients with UniV circulation.

Improvement in Interstage Survival in a National Pediatric Cardiology Learning Network.

Infants with univentricular congenital heart disease (CHD), including those with hypoplastic left heart syndrome (HLHS), regularly pose dilemmas in decision-making because their anatomy and physiology are often unique and variable. The typical staged surgical course for infants with complex univentricular anatomy with systemic outflow obstruction begins with the Norwood (stage 1) operation or variant shortly after birth, followed several months later by superior cavopulmonary anastomosis (stage 2 palliation) with an ultimate goal of a Fontan-type operation several years later.1–3 Improvement in surgical and postoperative management has led to considerable improvement in early post-Norwood survival in the recent era.4–7 However, after the Norwood procedure and before stage 2 palliation, a high-risk time period termed interstage, mortality has been previously been reported at 10% to 15%.8–10 The rare nature of this disorder has limited robust learning about successful strategies to improve survival undertaken by single-surgical centers, and a gap exists in our ability to further improve mortality in this population. The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), the first multicenter learning network within pediatric cardiology,11 was established with the goal of improving care and outcomes for children with univentricular heart after the Norwood operation and specifically to (1) improve interstage mortality, (2) decrease interstage growth failure, and (3) reduce interstage hospital readmissions for major medical events. There were several perceived challenges to success in changing clinical outcomes before starting the NPC-QIC collaborative. A primary challenge in collaboration among multiple sites can be agreement on best practices that should be implemented. This is especially true for rare diseases, such as univentricular heart disease, where evidence-based clinical guidelines are not available to clinicians. As noted above, major variation persists in management practices among individuals and institutions caring for children with HLHS and other …

Longitudinal development of obesity in the post-Fontan population.

An elevated body mass index (BMI) in childhood is a significant risk factor for cardiovascular disease, and it may pose an additional risk to children and adults with palliated univentricular congenital heart disease. However, little is known about longitudinal development of obesity in this population. The objective of this study is to determine the prevalence of overweight (OW) and obese (OB) habitus at the time of Fontan palliative surgery, to track changes in BMI after surgery, and ultimately to determine whether factors such as gender, ethnicity, preoperative heart defect and ventricular dominance are associated with later development of OW or OB. A retrospective chart review of 84 patients undergoing Fontan palliation was performed. Demographic data including gender, ethnicity, preoperative heart defect and ventricular dominance were recorded. Height, weight and BMI were obtained at the time of Fontan and on a yearly basis post surgery. At the time of Fontan palliation, 10.7% of patients were OB or OW. During the five years following palliation, the percentage of OB or OW patients trended upward, from 20.3% the year following surgery to 30% at 5 years post Fontan. Repeated measures generalized estimating equation showed a significant association between Hispanic ethnicity and increased BMI Z-scores for the 5 years after Fontan palliation (P<0.001); there was no association between BMI Z-scores and patient sex, lesion or ventricular dominance. During the first 5 years after Fontan palliation, there is a trend toward increasing percentages of OB and OW patients. In addition, there is a significant association between Hispanic ethnicity and being OW or OB before and after surgery. Further study is needed to determine whether OW/OB status is associated with worse health outcomes in this patient population.

Biomarkers of cardiac function and outcome in univentricular congenital heart disease

Biomarkers of cardiac function and outcome in univentricular congenital heart disease Harold S Bernstein,1,2 Hythem Nawaytou3 1Merck Sharp &amp; Dohme Corp, Whitehouse Station, NJ, USA; 2The Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA; 3Department of Pediatrics, University of California, San Francisco, CA, USA Abstract: A biomarker is a clinical characteristic that predicts the presence of a specific physiological state, degree of severity of a pathophysiological state, or long-term outcome resulting from that state. Broadly defined, a biomarker can be a clinical parameter, or the result of a biochemical assay or imaging measure. Children are imperfect historians and cardiac disease may mimic other organ system diseases. Hence, reliable biomarkers can greatly enhance diagnosis and management of pediatric cardiac disease. In this review, we discuss the use of biomarkers in managing complex congenital heart disease characterized by single ventricle physiology. Keywords: biomarker, natriuretic peptide, heart failure, congenital heart disease, single ventricle

Hepatology Highlights

Hepatology Highlights

Kasuistik – Fontan-Patient auf dem Southside Festival –60 000 Besucher / 119 999 Herzkammern

Patients with univentricular congenital heart disease are treated with Fontan-procedure for more than 40 years resulting in mainly good life-quality for pediatric and now more and more adult patients. Due to prolonged life expectation and adequate physical state, adolescent and adult Fontan-patients participate in normal life activities like the main population. Mass events like open-air concerts and sport events belong to these activities.Since 1999 the Southside Festival is one of the main open air events in southern Germany with about 60.000 visitors. Visitors with minor or major health problems are treated by emergency response services like the German Red Cross, the German St Johns Ambulance and the German Order of Malta. The article refers to the special pathophysiology and management of Fontan-patients in emergency and anaesthesiologic settings based on a case report from the Southside Festival. Due to increasing "fontanpopulation" profound education of attending physicians and paramedics is inevitable to provide good quality in prehospital and innerhospital care.

High sensitivity C-reactive protein: a biomarker for heart failure in children with univentricular heart disease

Children with univentricular congenital heart disease are at increased risk for devel- oping heart failure. However, detecting early heart failure in these patients is especially difficult because few objective measures have been validated in this cohort. The aim of this study is to determine the usefulness of high sensitivity C-reactive protein (hsCRP) as a biomarker for detecting heart failure in univentricular heart disease patients, and to define the impact of univen - tricular morphology and stage of palliation on its utility. A cross-sectional observational study of children with univentricular congenital heart disease aged 1 month to 7 years was conducted. The presence of heart failure was defined as a Ross score . 2. The association of hsCRP with heart failure was assessed using logistic regression and receiver operating characteristic curves. Of the 50 included children, 16 (32%) had clinical heart failure. A doubling of hsCRP resulted in a 0.31 unit increase in Ross score (95% confidence interval: -0.07, 0.56; P = 0.01) with a c-statistic of 68%. When stratified by ventricular morphology, the left univentricular morphology group exceeded the threshold of $75% (c-statistic = 81%) as did those at stage II of palliation (c-statistic = 85%). A cut point of $0.8 mg/mL correctly classified 75% of patients with left univentricular morphology, and 73% of patients regardless of morphology at stage II of palliation. From these results, we conclude that hsCRP is a useful biomarker of clinical heart failure in univentricular patients with left ventricular morphology at all stages of surgical palliation, and in all univentricular patients at stage II of palliation.

Re: Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome

[1] Tweddell JS, Hoffman GM, Mussatto KA, Fedderly RT, Berger S, Jaquiss RD et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002;106:I82–9. [2] Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ 3rd. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 2000;102:III136–41. [3] Furck AK, Uebing A, Hansen JH, Scheewe J, Jung O, Fischer G et al. Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey. J Thorac Cardiovasc Surg 2010;139:359–65. [4] Tweddell JS, Hoffman GS. Postoperative management in patients with complex congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:187–205. [5] McGuirk SP, Griselli M, Stumper OF, Rumball EM, Miller P, Dhillon R et al. Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart 2006;92:364–70. [6] Ghanayem NS, Hoffman GM, Mussatto KA, Cava JR, Frommelt PC, Rudd NA et al. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 2003; 126:1367–77. [7] Furck AK, Hansen JH, Uebing A, Scheewe J, Jung O, Kramer HH. The impact of afterload reduction on the early postoperative course after the Norwood operation—a 12-year single-centre experience. Eur J Cardiothorac Surg 2010;37:289–95. [8] Dobrolet NC, Nieves JA, Welch EM, Khan D, Rossi AF, Burke RP et al. New approach to interstage care for palliated high-risk patients with congenital heart disease. J Thorac Cardiovasc Surg 2011;142: 855–60. [9] Photiadis J, Hubler M, Sinzobahamvya N, Ovroutski S, Stiller B, Hetzer R et al. Does size matter? Larger Blalock-Taussig shunt in the modified Norwood operation correlates with better hemodynamics. Eur J Cardiothorac Surg 2005;28:56–60. [10] Ghanayem NS, Hoffman GM, Mussatto KA, Frommelt MA, Cava JR, Mitchell ME et al. Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 2010;140:857–63.

Plasma B‐type natriuretic peptide levels in children with heart disease

To determine plasma B-type natriuretic peptide (BNP) levels in children with heart diseases before medical or surgical treatment for monitoring therapeutic efficacy in an observational prospective clinical trial at tertiary care centre. In 522 paediatric patients at an age of 6.4 ± 5.2 years (mean ± SD; range: 14 days-18 years) with congenital heart disease (CHD), cardiomyopathies (CMP) or pulmonary arterial hypertension (PAH), plasma BNP levels were evaluated before and under treatment. Most types of heart disease are associated with increased mean plasma BNP levels before treatment, with highest values in children with CMP (BNP 6165 pg/mL in dilated CMP vs. 817 pg/mL in hypertrophic, vs. 1236 pg/mL in restrictive CMP, each p < 0.05). Children with PAH showed a significant decrease in BNP levels under medical treatment (mean BNP 981 pg/mL before vs. 26 pg/mL under treatment, p < 0.05). Children with univentricular CHD undergoing surgical staged palliation showed a significant decrease in BNP levels after bidirectional cavopulmonary anastomosis (BDCP) (BNP 109 pg/mL before vs. 70 pg/mL after BDCP, p < 0.05). Plasma BNP levels are elevated in children with heart disease before treatment and are a useful laboratory parameter under treatment during long-term follow-up.

Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease

Survival of high-risk patients with univentricular heart disease after Norwood palliation is reduced. We hypothesized that early goal-directed monitoring with venous oximetry and near-infrared spectroscopy would offset their increased vulnerability and improve survival. A prospective database of patients undergoing stage 1 palliation was used to assess differences in outcomes across risk groups in the setting of a comprehensive, goal-directed monitoring program. High-risk criteria included gestational age 35 weeks or less, birth weight less than 2.5 kg, and additional cardiac or extracardiac anomalies. Outcomes included survival to defined end points and measures of postoperative support. From September 2000 to September 2008, 162 patients underwent stage 1 palliation: 28% (45/162) high-risk and 72% (117/162) standard-risk patients. Lesions other than hypoplastic left heart syndrome were more common among high-risk patients (38%, 17/45, vs 15%, 18/117, P = .003). Operative survival was not statistically different(87%, 39/45, high risk vs 95%, 111/117, standard risk, P = .1). High-risk patients were more likely to receive inpatient treatment until stage 2 palliation (24%, 11/45, vs 10%, 12/117, P = .001) and had lower 1-year survival (78% vs 93%, P = .01) and survival to date (71% vs 92%, P = .001). Intensive monitoring partially offset biologic vulnerability of high-risk patients, helping attain comparable early outcomes. Vulnerability persisted throughout the interstage period, however, and increased mortality beyond cavopulmonary shunt was seen only among high-risk patients. Although enhanced monitoring reduced early mortality, high resource use and attrition after stage 2 palliation suggest an ongoing need to evaluate our current palliative strategy for this subset of patients.

B-type Natriuretic Peptide: Perioperative Patterns in Congenital Heart Disease

B-type natriuretic peptide (BNP) has diagnostic, prognostic, and therapeutic roles in adults with heart failure. BNP levels in children undergoing surgical repair of congenital heart disease (CHD) were characterized broadly, and distinguishable subgroup patterns delineated. Prospective, blinded, observational case series. Academic, tertiary care, free-standing pediatric hospital. Children with CHD; controls without cardiopulmonary disease. Interventions. None. Preoperative cardiac medications/doses, CHD lesion types, perioperative BNP levels, intraoperative variables (lengths of surgery, bypass, cross-clamp), postoperative outcomes (lengths of ventilation, hospitalization, open chest; averages of inotropic support, central venous pressure, perfusion, urine output; death, low cardiac output syndrome (LCOS), cardiac arrest; readmission; and discharge medications). Median BNP levels for 102 neonatal and non-neonatal controls were 27 and 7 pg/mL, respectively. Serial BNP measures from 105 patients undergoing CHD repair demonstrated a median postoperative peak at 12 hours. The median and interquartile postoperative 24-hour average BNP levels for neonates were 1506 (782-3784) pg/mL vs. 286 (169-578) pg/mL for non-neonates (P < 0.001). Postoperative BNP correlated with inotropic requirement, durations of open chest, ventilation, intensive care unit stay, and hospitalization (r = 0.33-0.65, all P < 0.001). Compared with biventricular CHD, Fontan palliations demonstrated lower postoperative BNP (median 150 vs. 306 pg/mL, P < 0.001), a 3-fold higher incidence of LCOS (P < 0.01), and longer length of hospitalization (median 6.0 vs. 4.5 days, P= 0.01). Perioperative BNP correlates to severity of illness and lengths of therapy in the CHD population, overall. Substantial variation in BNP across time as well as within and between CHD lesions limits its practical utility as an isolated point-of-care measure. BNP commonly peaks 6-12 hours postoperatively, but the timing and magnitude of BNP elevation demonstrates notable age-dependency, peaking earlier and rising an order of magnitude higher in neonates. In spite of higher clinical acuity, non-neonatal univentricular CHD paradoxically demonstrates lower BNP levels compared with biventricular physiologies.

Incorporation of the Hepatic Veins Into the Cavopulmonary Circulation in Patients With Heterotaxy and Pulmonary Arteriovenous Malformations After a Kawashima Procedure

In patients with polysplenia syndrome and azygous continuation of an interrupted inferior vena cava (IVC), pulmonary arteriovenous malformations (PAVMs) are relatively common after bidirectional cavopulmonary anastomosis (BCPA, Kawashima procedure). Resolution of PAVMs after hepatic vein (HV) inclusion into the cavopulmonary circulation has been reported, but there has been no systematic investigation of the effects of this therapy in a population of more than 3 patients. We studied 16 patients with heterotaxy, univentricular congenital heart disease, and azygous continuation of the IVC who underwent incorporation of the HV into the cavopulmonary circuit for treatment of significant PAVMs after a Kawashima procedure. The median preoperative systemic arterial oxygen saturation (SsaO2) was 76% (65%-85%), compared with 89% (85% to 92%) early after BCPA. Among 15 early survivors, the median early postoperative SsaO2 was 76% (56%-85%). In 11 of the 15 survivors, SsaO2 rose to 90% or greater within a year and remained at 93% or greater at follow-up of 2.8 to 10 years. Four patients had persistent hypoxemia and residual PAVMs at follow-up catheterization 1.5 to 8 years postoperatively; these patients had the most severe hypoxemia prior to HV inclusion, and in 2 the residual PAVMs were unilateral, with HV flow streaming to the contralateral lung, in which PAVMs had resolved. Hypoxemia resolved after cavopulmonary incorporation of the HV in the majority of our patients with PAVMs after the Kawashima operation, presumably due to a combination of PAVM resolution and elimination of hepatic venoatrial right-to-left shunting. These findings support the theory that development of PAVMs is facilitated by exclusion of HV effluent from the pulmonary circulation.

Maturational and hemodynamic factors predictive of increased cyanosis after bidirectional cavopulmonary anastomosis

Bidirectional cavopulmonary anastomosis (BCA) is thought to be beneficial in the palliation of patients with univentricular congenital heart disease considered at high risk for Fontan repair. Experience with patients undergoing BCA suggested that those who were older or larger at the time of surgery tended to be more cyanotic postoperatively than their younger and smaller counterparts. This study was designed to identify correlates of systemic arterial oxygen saturation after BCA. Specifically, it was postulated that maturational changes in blood flow distribution might be associated with decreasing arterial oxygen saturation. Database records of all 110 patients undergoing BCA at our institution from June 1988 until the end of 1991 were reviewed. Postoperative catheterization data were available for 66 patients. Twenty-one patients were excluded because they had potentially reversible causes of cyanosis yielding inestimable degrees of error in hemodynamic calculations. In the remaining 45 patients, univariate and multivariate regression analyses were used to identify correlates of systemic arterial oxygen saturation. Growth and maturation as represented by body surface area exhibited a highly significant inverse correlation with arterial oxygen saturation (p = 0.005), as did pulmonary vascular resistance (p = 0.003). Patients who underwent BCA when > 3.9 years of age or with body surface area > 0.65 m2 were at significantly increased risk for excessive postoperative cyanosis, defined as systemic arterial oxygen saturation < 75% (p < 0.005). The interval between surgery and catheterization correlated directly with arterial oxygen saturation (p = 0.002), indicating a tendency toward earlier study of more cyanotic patients. None of the other variables examined correlated significantly with arterial oxygen saturation. Patient age and body surface area were associated with postoperative desaturation independently of other known causes of cyanosis. Our findings suggest that BCA is optimally performed as interim palliation in young patients who may be expected to become suitable candidates for Fontan repair.

Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy

Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty-one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, x 2 testing) showed that neither the presence of TAPVD ( p = 0.7) nor TAPVD repair alone ( p = 0.8) or with shunting ( p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality ( p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated.