L. Reardon

Novel techniques of mechanical circulatory support for the right heart and Fontan circulation

BACKGROUND Currently available ventricular assist devices are designed primarily for use in patients with left sided heart failure. This study evaluated the efficacy of the Jarvik 2000 ventricular assist device (VAD) as a pulmonary pump to power a Fontan circuit in a large animal model. METHODS Without the use of cardiopulmonary bypass, Fontan circulations were surgically created in 4 pigs (50 kg) using synthetic grafts from the inferior and superior vena cavas to the main pulmonary artery. Subsequently, the VAD was implanted within the common Fontan graft to provide a pulmonary pump. Direct chamber pressures and epicardial Doppler images were taken during the various phases of the experiment. Heart rate, femoral artery blood pressure, oxygen saturation, and aortic flow rate were continuously recorded. The outflow cannula of the VAD was then partially banded by 50% and then 75% to mimic increased afterload. RESULTS Fontan and VAD implantation was successfully performed in all 4 animals. Arterial pressure and aortic flow decreased dramatically with institution of the Fontan but were restored to baseline upon activation of the VAD. The pressure within the systemic venous circulation rose precipitously with institution of the Fontan circulation and improved appropriately with activation of the VAD. Adequate perfusion was maintained during increased afterload. CONCLUSIONS An axial flow VAD can restore normal hemodynamics and cardiac output when used as a pulmonary pump in a Fontan circulation. A VAD can rescue a failing Fontan as a bridge to transplant or recovery, even in the setting of high pulmonary resistance.

Usefulness of Serum Brain Natriuretic Peptide to Predict Adverse Events in Patients With the Eisenmenger Syndrome

The aim of this study was to evaluate the prognostic value of brain natriuretic peptide (BNP) in outpatients with the Eisenmenger syndrome (ES). BNP is often elevated in patients with cyanotic congenital heart disease. The clinical utility of BNP in patients with cyanotic congenital heart disease and the ES has not been clearly delineated. Records of adults with ES who had undergone serum BNP measurement were reviewed. The primary end point was death or heart failure admission. Fifty-three patients were included, with 15 patients (28%) meeting the primary end point (death in 7, heart failure hospitalization in 8). Mean and median baseline BNP in patients meeting the primary end point were 322 ± 346 and 179 pg/ml, compared to 100 ± 157 and 41 pg/ml in those not meeting the primary end point (p = 0.0029). A Cox proportional-hazards model using baseline BNP between the 2 groups yielded a hazard ratio of 1.84 (95% confidence interval [CI] 1.19 to 2.85, p = 0.006). The relative risk for baseline BNP level >140 pg/ml was 4.62 (95% CI 1.80 to 11.3, p = 0.008). Patients who met the primary end point increased their BNP levels by 42.5 pg/ml per year (95% CI 12.09 to 72.95, p = 0.006) compared to 7.2 pg/ml per year (95% CI 2.01 to 12.47, p = 0.007) in patients who did not meet the primary end point. In conclusion, elevated BNP levels are predictive of death or heart failure admission in patients with the ES. A serum BNP level >140 pg/ml is a useful tool in identifying high-risk patients.

An Observation from Liver Biopsies Two Decades Post-Fontan

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy.

A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings

OBJECTIVE We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan. METHODS Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the number of years post-Fontan. Further, we analyzed correlations between Fontan hepatic index values and fibrosis scores from hepatic biopsy. RESULTS We identified a total of 79 post-Fontan patients that underwent cardiac catheterization and liver biopsy. Of the 79 patients, 53 met inclusion criteria, and 32 consented to undergo hepatic-ultrasound elastography. Of the 32 that underwent elastography, data from 30 patients was used for analysis. We found no statistically significant differences in demographics, laboratory values, or cardiac catheterization data between the 30 included patients and the 21 that did not participate. Utilizing data from the 30 included patients, we found a strong, highly statistically significant correlation between the Fontan hepatic index values and total fibrosis scores (R = 0.8, P < .00001). However, the cohort size prevented reliable discriminating cut-off values for the range of total fibrosis scores. CONCLUSIONS In a small cohort of patients post-Fontan, preliminary findings suggest that the composite Fontan hepatic index might be a clinically useful, noninvasive method of serially monitoring for hepatic fibrosis. Further studies, with large patient cohorts, are necessary to validate our findings and develop clinically useful discriminatory cutoff values.

Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient.

We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease. A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset.

Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.

Prognostic utility of MELD-XI in adult congenital heart disease patients undergoing cardiac transplantation

Model of End‐Stage Liver Disease eXcluding INR (MELD‐XI) at cardiac transplant has demonstrated prognostic survival utility, but has not been specifically validated in adult congenital heart disease (ACHD) in a registry study.

Heart and heart-liver transplantation in adults with failing Fontan physiology

As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart–liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients has improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology.

TRANSITION TO ADULTHOOD: HEART TRANSPLANT (HT) RECIPIENT OUTCOMES BY AGE GROUP

Outcomes of post-HT patients in transition (13y to 24y) have not been well described. 45213 HT recipients were identified from UNOS (1987-2011) and stratified by age group (pediatric (age 24, n=38322). re-HT & lost to follow up. Survival was censored at 12y. Multivariate Cox proportional hazard

Heart Failure and CardiomyopathiesINFLUENCE OF MELD (MODEL OF END-STAGE LIVER DISEASE)_XI (EXCLUDING INR) ON PEDIATRIC POST-HEART TRANSPLANT (HT) OUTCOMES

Liver dysfunction increases post-surgical morbidity and mortality. MELD-XI has been evaluated in ambulatory heart failure patients and in those receiving VAD support in a single center. Use of MELD-XI in a pediatric post-HT population has not been previously reported. 5711 HT recipients were