Determinants and Prognostic Implications of Hepatorenal Dysfunction in Adults With Congenital Heart Disease.

BACKGROUND: There are limited data on the prognostic role of hepatorenal function indices in ambulatory patients with congenital heart disease (CHD). The purpose of this study was to determine the prevalence, risk factors, and prognostic implications of hepatorenal dysfunction, as measured by Model for End-Stage Liver Disease Excluding International Normalised Ratio (MELD-XI) score, in adults with CHD. METHODS: In this retrospective study of CHD patients with comprehensive metabolic panels (2003-2019), mild/moderate and severe hepatorenal dysfunction was defined as MELD-XI 11-15 and > 15, respectively. RESULTS: Of 4977 patients, 1376 (28%) had hepatorenal dysfunction (mild/moderate: n = 935 [19%]; severe: n = 441 [9%]). Hepatorenal dysfunction was most common in Fontan/unrepaired single ventricle (46%) and right heart disease (31%). Baseline MELD-XI was associated with all-cause mortality (HR 1.27, CI 1.21-1.33; P < 0.001) after adjustment for age, sex, and congenital heart lesion. In 3864 patients with serial MELD-XI data, there was a temporal increase in MELD-XI, and this was associated with an increased risk of mortality (HR 1.24, CI 1.15-1.36, per unit increase in MELD-XI; P = 0.004), independently from the baseline MELD-XI score. In the subset of 1856 patients that underwent surgical/transcatheter interventions, there was a postoperative reduction in MELD-XI, and this was associated with a lower risk of mortality (HR 0.94, CI 0.90-0.98, per unit decrease in MELD-XI; P = 0.008), independently from the baseline MELD-XI score. CONCLUSIONS: Hepatorenal dysfunction was common in adults with CHD. Both baseline MELD-XI score and temporal changes in MELD-XI were associated with clinical outcomes, and therefore could be used to monitor therapeutic response to interventions and for deterioration in clinical status.

Improvement in hepatic and renal function following isolated heart transplant in adults with congenital heart disease.

BACKGROUND: There are limited data about postoperative changes in hepatic and renal function in adults with congenital heart disease (CHD) undergoing isolated heart transplant. The purpose of this study was to assess postoperative changes in hepatic and renal function in this population. METHODS: Retrospective cohort study of adults with CHD undergoing isolated heart transplant at Mayo Clinic (2003-2019). Global hepatic function was assessed using the model for end-stage liver disease excluding international normalized ratio [MELD-XI]) score; hepatic fibrosis was assessed using the fibrosis-4 (FIB-4) score and aspartate/platelet ratio index (APRI); and renal function was assessed using estimated glomerular filtration rate (GFR). All indices were measured preoperatively and postoperatively (at 6 months, 1 year, 2 years). RESULTS: Of 40 patients (age 41 ± 8 years) in the study, 35 had complete preoperative and postoperative data. There was a temporal improvement in hepatic and renal indices from preop (MELD-XI 14 ± 5, APRI 0.60 ± 0.23, FIB-4 1.44 ± 0.38, GFR 59 [44-83]) to 6 months postop (MELD-XI 12 ± 6, APRI 0.49 ± 0.17, FIB-4 1.29 ± 0.33, GFR 68 [54-96]) and 1-year postop (MELD-XI 9 ± 3, APRI 0.41 ± 0.16, FIB-4 1.12 ± 0.29, GFR 82 [69-108]), p < 0.05 for all comparisons. CONCLUSIONS: CHD patients undergoing isolated heart transplant had significant improvement in hepatic and renal function. These data suggests that selected CHD patients may do well with isolated heart transplant despite reduced hepatic and renal function, and hepatic fibrosis preoperatively. More rigorous prospective studies are required to determine the relative outcomes of isolated versus combined heart-liver transplant in this population.

Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.

AIMS: Conotruncal anomalies share common embryogenic defects of the outflow tracts and great arteries, which result in a predisposition to aortic aneurysms. The purpose of this study was to describe the prevalence and risk of progressive aortic aneurysms in adults with conotruncal anomalies. METHODS AND RESULTS: Retrospective study of adults with conotruncal anomalies that underwent cross-sectional imaging 2003-20. Aneurysm was defined as aortic root/mid-ascending aorta >2.1 mm/m2/>1.9 mm/m2, progressive aneurysm as increase by >2 mm, and severe aneurysm as dimension >50 mm. Of 2261 patients (38 ± 12 years; male 58%), 1167 (52%) had an aortic aneurysm, and 205 (14%) had a severe aortic aneurysm. Mean annual increase in aortic root/mid-ascending aorta was 0.3 ± 0.1 mm/0.2 ± 0.1 mm. The 3-, 5-, and 7-year cumulative incidence of the progressive aortic aneurysm was 4%, 7%, and 9%, respectively. The rate of aneurysm growth decreased with age, with no significant growth after age 40 years. There was an excellent correlation between aortic indices from cross-sectional imaging and echocardiography. Of 950 females, 184 had ≥1 pregnancy, and 81 (44%) of the 184 patients had aortic aneurysm prior to pregnancy. There was no aortic dissection or progression of the aortic aneurysm during pregnancy. Overall, there was no aortic dissection during 7984 patient-years of follow-up. CONCLUSIONS: Aortic aneurysm was common in patients with conotruncal anomalies. However, the risk of progressive aneurysm or dissection was low. Collectively, these data suggest a benign natural history and perhaps a less frequent need for cross-sectional imaging. Further studies are required to determine the optimal timing for surgical intervention in this population.

Determinants of Aerobic Capacity After Tricuspid Valve Replacement in Congenitally Corrected Transposition of Great Arteries.

Background: Systemic tricuspid regurgitation and impaired aerobic capacity are common in adults with congenitally corrected transposition of great arteries (cc-TGA), but it is unknown whether systemic tricuspid valve replacement (sTVR) is associated with improvement in aerobic capacity. Objectives: The purpose of this study was to determine whether sTVR was associated with postoperative improvement in aerobic capacity as measured by peak oxygen consumption (VO2). Methods: This is a retrospective cohort study of adults with cc-TGA and ≥moderate tricuspid regurgitation that underwent sTVR and exercise test pre- and post-sTVR (2003-2019). We selected a propensity-matched control group of patients with cc-TGA and ≥moderate tricuspid regurgitation that did not undergo sTVR between exercise tests. Improvement in aerobic capacity was calculated as the difference between baseline and follow-up exercise tests. Results: The study comprised 48 patients in each group, and both groups had similar peak VO2 at baseline. Compared to the baseline exercise test, the sTVR group had more improvement in peak VO2 (6 ± 4% vs -8 ± 3%-predicted, P = 0.001), and the factors associated with postoperative improvement in peak VO2 were baseline systolic blood pressure (<120 mm Hg), systemic right ventricular global longitudinal strain (<-16%), and preoperative peak VO2 (>60%-predicted). Conclusions: There was an improvement in aerobic capacity after sTVR in patients with cc-TGA and severe tricuspid regurgitation. The determinants of improvement in aerobic capacity were baseline systolic blood pressure, systemic right ventricular global longitudinal strain, and preoperative peak VO2. Further studies are required to determine whether strict blood pressure control and optimal timing of sTVR based on these indices would result in improved clinical outcomes in this population.

A Spectrum of Pulmonary Complications Occurring in End-Stage Renal Disease Patients on Maintenance Hemodialysis.

Objective To investigate the trends of end-stage renal disease (ESRD) in patients undergoing maintenance hemodialysis (MHD) and find the correlation with effects on the pulmonary system in such patients. Methodology A multicentric prospective study was conducted in the city of Solapur, India. Data were collected from 250 patients through interpersonal interrogation using a questionnaire to capture basic demographic details, the history of ESRD, and relevant respiratory symptoms like breathlessness, cough, fever, etc. related to their disease. Symptoms that are likely associated with the pulmonary system were analyzed and referred to the pulmonology department. Appropriate diagnoses were made using relevant diagnostic tools like X-rays and sputum studies. The association between various disease attributes and pulmonary diagnoses was analyzed using the chi-square (χ2) test, with a p-value of value less than or equal to 0.05 considered statistically significant. Various socio-demographic variables, existing comorbidities, occupation-related risk factors, smoking history, past or current history of any respiratory conditions, the association between the causes of ESRD, time since the first dialysis and sociodemographic factors, and frequency of pulmonary complications were the other covariates in the study. Results Our study reports that 31.6% of our patients had significant impairment in their functioning due to respiratory complaints. The prevalence of respiratory complications was 27.2%. Major contributors were pleural effusion (33.8), pneumonia (25), pulmonary edema (20.58), pleuritis (11.76), collapse (8.8), tuberculosis (5.8), fibrosis (4.4), pericardial effusion (4.4), calcification (2.9), and hydrothorax (1.47). We report one case of Urinothorax as a rare cause of hydrothorax in such patients. Overall, our analysis found a significant association between non-reporting of respiratory complaints and acute admissions to the intensive care unit (ICU) with a respiratory cause at p-value 0.0076 with a greater predilection toward the rural populations. Conclusion Our study results highlight the prevalence of pulmonary complications in ESRD patients. The occurrence of pulmonary complications, irrespective of the presence of symptoms and a greater association between non-reporting of respiratory symptoms and acute admissions to the ICU, is a hallmark to consider the importance of history and clinical vigilance during patient visits.