Relief of branch pulmonary artery stenosis reduces pulmonary valve insufficiency in a swine model

ObjectiveWe sought to determine the impact of relieving branch pulmonary artery stenosis on pulmonary valve insufficiency and right ventricular function. Long-standing pulmonary insufficiency causes progressive right ventricular dilatation, leading to decreased right ventricular function. Adults with pulmonary insufficiency are at risk of decreased exercise tolerance, arrhythmias, and sudden cardiac death. Branch pulmonary artery stenosis frequently occurs in these patients, and the presence of branch stenosis may exacerbate valve insufficiency.MethodsNeonatal piglets (n = 7) underwent surgery to create pulmonary insufficiency and left pulmonary artery stenosis. At 3 months of age, the animals underwent baseline cardiac magnetic resonance imaging followed by stenting of the left pulmonary artery. A repeat magnetic resonance imaging scan was performed 1 week after intervention. Magnetic resonance imaging evaluation included (1) velocity mapping to assess the forward and reverse flow at the main, left and right pulmonary arteries, and aorta; and (2) volumetric assessment of the right ventricle.ResultsLeft pulmonary artery flow increased from 14.5% to 36.3% of total net flow after stenting (P < .01). Pulmonary regurgitation decreased from 38.7% to 27.4% (P < .02). Right ventricular ejection fraction improved from a median of 53.5% to 58.2% after stenting (P < .01). Cardiac index improved from a median of 2.7 to 3.5 L/min/m2 (P = .01).ConclusionRelief of branch pulmonary artery stenosis reduces insufficiency and improves right ventricular systolic function in this animal model. This supports the practice of aggressive intervention in patients with branch pulmonary artery stenosis and pulmonary insufficiency

Anomalous Aortic Origin of a Coronary Artery With an Interarterial Course Should Family Screening Be Routine?

ObjectivesWe sought to present cases of familial occurrence of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA) to determine if it would alter our current screening and management recommendations.BackgroundAnomalous aortic origin of a coronary artery with an interarterial course is a rare congenital anomaly that carries an increased risk of sudden death in children and young adults. There are no reports in the literature of familial AAOCA in the pediatric population.MethodsIn preparation for a multi-institutional prospective study evaluating patient management and surgical outcomes in children and young adults with AAOCA, a questionnaire was sent to multiple pediatric institutions in North and South America. Several respondents indicated caring for families with more than 1 member with AAOCA. These patients were identified and charts were retrospectively reviewed.ResultsWe identified 5 families in which a child was diagnosed with AAOCA and another family member was subsequently identified through screening with echocardiography. The odds of this occurring are significantly greater than what would be expected by chance. All identified by screening were asymptomatic and had anomalous right coronary artery despite 2 of the 5 index cases having anomalous left coronary artery.ConclusionsIt is possible that there is a genetic link for AAOCA. Future research into this is warranted. Due to the potential risk of myocardial ischemia and sudden death associated with AAOCA, screening first-degree relatives for AAOCA using transthoracic echocardiography would be the prudent approach to potentially prevent a sudden catastrophic event

Genetic factors are important determinants of impaired growth after infant cardiac surgery

ObjectivesWe sought to estimate the prevalence and identify the predictors of impaired growth after infant cardiac surgery.MethodsWe performed a secondary analysis of a prospective study of the role of apolipoprotein E gene polymorphisms on neurodevelopment in young children after infant cardiac surgery. Prevalence estimates for growth velocity were derived by using anthropometric measures (weight and head circumference) obtained at birth and at 4 years of age. Genetic evaluation was also performed. Growth measure z scores were calculated by using World Health Organization Child Growth Standards. Growth velocity was evaluated by using 2 different techniques: first by clustering the children into one of 3 growth velocity subgroups based on z scores (impaired growth, difference < −0.5 standard deviation; stable growth, difference of −0.5 to 0.5 standard deviation; and improving growth, difference > 0.5 SD) and second by using continuous difference scores. Statistical analyses were conducted with a combination of proportional odds models for the ordered categories and simple linear regression for the continuous outcomes.ResultsThree hundred nineteen full-term subjects had complete anthropometric measures for weight and head circumference at birth and 4 years. The cohort was 56% male. Genetic examinations were available for 97% (309/319) of the cohort (normal, 74%; definite or suspected genetic abnormality, 26%). Frequency counts for weight categories were as follows: impaired growth, 37%; stable growth, 31%; and improving growth, 32%. Frequency counts for head circumference categories were as follows: impaired growth, 39%; stable growth, 28%; and improving growth, 33%. The presence of a definite or suspected genetic syndrome (P = .04) was found to be a predictor of impaired growth for weight but not for head circumference. When growth z scores were used as continuous outcomes, the apolipoprotein E ε2 allele was found to be predictive of lower z scores for both weight (P = .02) and head circumference (P = .03).ConclusionsImpaired growth for both weight and head circumference is common (both >30%) in this cohort of children after infant cardiac surgery. Both the apolipoprotein E ε2 allele and the presence of a definite or suspected genetic syndrome were associated with impaired weight growth velocity. The apolipoprotein E ε2 allele was also associated with impaired growth velocity for head circumference. Persistent poor growth might have long-term implications for the health and development of children with congenital heart defects

Chronic hypoxemia increases myocardial cytochrome oxidase

ObjectiveCyanotic patients have potentially decreased tissue oxygen tension. Cytochrome oxidase catalyzes the reduction of oxygen and is integral to adenosine triphosphate production. Cytochrome oxidase subunit I, the active site, is encoded by mitochondrial DNA. Using a newborn swine model of chronic hypoxemia, we evaluated ventricular cytochrome oxidase subunit I mRNA and protein expression and assessed cytochrome oxidase activity.MethodsThirty-two newborn piglets underwent thoracotomy and placement of a pulmonary artery–to–left atrium shunt or sham operation. Two weeks later, partial pressure of arterial oxygen, hematocrit, and left ventricular shortening fraction values were compared with baseline values. Northern blot hybridization and protein immunoblotting for ventricular cytochrome oxidase subunit I were performed. Cytochrome oxidase kinetic activity was measured. Heme a,a3 content and turnover number were determined. Significance was assessed with a t test.ResultsBaseline partial pressure of arterial oxygen and hematocrit values were similar. Hypoxemic piglets had a lower partial pressure of arterial oxygen of 38 ± 10 mm Hg (P < .001) and higher hematocrit value of 31.4% ± 2.9% (P < .001) compared with a partial pressure of arterial oxygen of 140 ± 47 mm Hg and hematocrit value of 24.6% ± 3.9% after the sham operation. Baseline and postprocedure left ventricular shortening fraction were similar within and between groups. Chronic hypoxemia increased right ventricular and left ventricular cytochrome oxidase I mRNA and protein by more than 1.4-fold. Cytochrome oxidase activity increased significantly in hypoxemia by 2.5-fold compared with that seen after the sham operation. Heme a,a3 content and turnover number increased by 1.5-fold during hypoxemia.ConclusionsChronic hypoxemia increases cytochrome oxidase I message, protein expression, and activity. The increase in kinetics was due to increased enzyme content and catalytic activity. This is a possible adaptive mechanism that might preserve organ function during chronic hypoxemia

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction

ObjectiveWe compare the hemodynamics and perioperative course of shunt type in hypoplastic left heart syndrome at the time of stage 2 reconstruction and longer-term survival.MethodsWe retrospectively reviewed the echocardiograms, catheterizations, and hospital records of all patients who had a stage 1 reconstruction between January 2002 and May 2005 and performed a cross-sectional analysis of hospital survivors.ResultsOne hundred seventy-six patients with hypoplastic left heart syndrome and variants underwent a stage 1 reconstruction with either a right ventricle–pulmonary artery conduit (n = 62) or a modified Blalock–Taussig shunt (n = 114). The median duration of follow-up is 29.1 months (range, 0-57 months). By means of Kaplan–Meier analysis, there is no difference in survival at 3 years (right ventricle–pulmonary artery conduit: 73% [95% confidence limit, 59%–83%] vs modified Blalock–Taussig shunt: 69% [95% confidence limit, 59%–77%]; P = .6). One hundred twenty-four patients have undergone stage 2 reconstruction (78 modified Blalock–Taussig shunts and 46 right ventricle–pulmonary artery conduits). At the time of the stage 2 reconstruction, patients with right ventricle–pulmonary artery conduits were younger (153 days [range, 108–340 days]; modified Blalock–Taussig shunt, 176 days [range, 80–318 days]; P = .03), had lower systemic oxygen saturation (73% [range, 58%–85%] vs 77% [range, 57%–89%], P < .01), and had higher preoperative hemoglobin levels (15.8 g/dL [range, 13–21 g/dL] vs 14.8 g/dL [range, 12–19 g/dL], P < .01) compared with those of the modified Blalock–Taussig shunt group. By means of echocardiographic evaluation, there was a higher incidence of qualitative ventricular dysfunction in patients with right ventricle–pulmonary artery conduits (14/46 [31%] vs 9/73 [12%], P = .02). However, no difference was observed in common atrial pressure or the arteriovenous oxygen difference.ConclusionInterim analyses suggest no advantage of one shunt type over another. This report raises concern of late ventricular dysfunction and outcome in patients with a right ventricle–pulmonary artery conduit

Modified ultrafiltration improves cerebral metabolic recovery after circulatory arrest

AbstractModified ultrafiltration uses hemofiltration of the patient and bypass circuit after separation from cardiopulmonary bypass to reverse hemodilution and edema. This study investigated the effect of modified ultrafiltration on cerebral metabolic recovery after deep hypothermic circulatory arrest. Twenty-six 1-week-old piglets (2 to 3 kg) were supported by cardiopulmonary bypass (37° C) at 100 ml · kg-1 · min-1 and cooled to 18° C. Animals underwent 90 minutes of circulatory arrest followed by rewarming to 37° C. After being weaned from cardiopulmonary bypass, animals were divided into three groups: controls ( n = 10); modified ultrafiltration for 20 minutes ( n = 9); transfusion of hemoconcentrated blood for 20 minutes ( n = 7). Global cerebral blood flow was measured by xenon 133 clearance methods: stage I--before cardiopulmonary bypass; stage II—5 minutes after cardiopulmonary bypass; and stage III—25 minutes after cardiopulmonary bypass. Cerebral metabolic rate of oxygen consumption, cerebral oxygen delivery, and hematocrit value were calculated for each time point. At point III, the hematocrit value (percent) was elevated above baseline in the ultrafiltration and transfusion groups (44 ± 1.8, 42 ± 1.8 versus 28 ± 1.7, 30 ± 0.7, respectively, p < 0.05). Cerebral oxygen delivery (ml · 100 gm-1 · min-1 ) increased significantly above baseline at point III after ultrafiltration (4.98 ± 0.32 versus 3.85 ± 0.16, p < 0.05) or transfusion (4.59 ± 0.17 versus 3.89 ± 0.06, p < 0.05) and decreased below baseline in the control group (2.77 ± 0.19 versus 3.81 ± 0.16, p < 0.05). Ninety minutes of deep hypothermic circulatory arrest resulted in impaired cerebral metabolic oxygen consumption (ml · 100 gm-1 · min-1 ) at point III in the control group (1.95 ± 0.15 versus 2.47 ± 0.07, p < 0.05) and transfusion group (1.72 ± 0.10 versus 2.39 ± 0.15, p < 0.05). After modified ultrafiltration, however, cerebral metabolic oxygen consumption at point III had increased significantly from baseline (3.12 ± 0.24 versus 2.48 ± 0.13, p < 0.05), indicating that the decrease in cerebral metabolism immediately after deep hypothermic circulatory arrest is reversible and may not represent permanent cerebral injury. Use of modified ultrafiltration after cardiopulmonary bypass may reduce brain injury associated with deep hypothermic circulatory arrest. (J THORAC CARDIOVASC SURG 1995;109:744-52

Hypoplastic Left Heart Syndrome Current Considerations and Expectations

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients

Development and Validation of a Seizure Prediction Model in Neonates Following Cardiac Surgery

BACKGROUND Electroencephalographic seizures (ES) following neonatal cardiac surgery are often subclinical and have been associated with poor outcomes. An accurate ES prediction model could allow targeted continuous electroencephalographic monitoring (CEEG) for high-risk neonates. METHODS Development and validation of ES prediction models in a multi-center prospective cohort where all postoperative neonates with cardiopulmonary bypass (CPB) underwent CEEG. RESULTS ES occurred in 7.4% of neonates (78 of 1053). Model predictors included gestational age, head circumference, single ventricle defect, DHCA duration, cardiac arrest, nitric oxide, ECMO, and delayed sternal closure. The model performed well in the derivation cohort (c-statistic 0.77, Hosmer-Lemeshow p=0.56), with a net benefit (NB) over monitoring all and none over a threshold probability of 2% in decision curve analysis (DCA). The model had good calibration in the validation cohort (Hosmer-Lemeshow, p=0.60); however, discrimination was poor (c-statistic 0.61) and in DCA there was no NB of the prediction model between the threshold probabilities of 8% and 18%. Using a cut-point that emphasized negative predictive value (NPV) in the derivation cohort, 32% (236 of 737) of neonates would not undergo CEEG, including 3.5% (2 of 58) with ES (NPV 99%, sensitivity 97%). CONCLUSIONS In this large prospective cohort, a prediction model of ES in neonates following CPB had good performance in the derivation cohort with a NB in DCA. However, performance in the validation cohort was weak with poor discrimination, calibration, and no NB in DCA. These findings support CEEG monitoring of all neonates following CPB