ABSTRACT
Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation. Methods: A retrospective analysis was conducted on patients who underwent HTX for congenital heart disease. Patients were categorized into two groups based on the pre-HTX circulation pathway: the Failing Fontan Group (FFG) and the Biventricular Congenital Group (BCG). Data were collected from patients between 1987 and 2018. Early and late outcomes, including survival rates, were assessed and critically analyzed. Results: Of the 66 patients, 29 (43%) had a failing Fontan palliation (FFG), and 37 had biventricular congenital diseases (BCG) before heart transplantation. Early mortality (30-day) was not statistically different between the two group. The overall survival rate was 82.6 ± 13.9% at 1 year, 79.0 ± 14.9% at 5 years, 67.2 ± 17.6% at 10 years and 63.2 ± 18.2 ± at 15 years for the FFG, and 86.1 ±11.4% at 1 year, 79.5 ± 13.7% at 5 years, 75.7 ± 14.9% at 10 years, 75.7 ± 14.9% at 15 years for the BCG, with no statistically significant difference (Mantel Cox p value: 0.69, 0.89, 0.52 and 0.39, respectively). Regarding Cox-regression analysis, the long-term survival rate was not affected either by previous Fontan surgery or by the era of heart transplantation (before vs. after the year 2000). Conclusions: Although heart transplantation after Fontan palliation showed a higher risk in the early post-operative period, the medium- and long-term survival rates are comparable with biventricular circulation patients. Despite the failing Fontan patients being a challenging set of candidates for transplantation, it is a reasonable option in their treatment.
ABSTRACT
Cor triatriatum dexter (CTD) is an extremely uncommon and underreported congenital cardiac anomaly in which the persistence of the embryonic right venous valve separates the right atrium into two chambers with varying degrees of obstruction to antegrade flow and variable degree of right to left shunt at atrial level. Depending on the size of the valves, clinical manifestations vary from absence of symptoms to severe hypoxia, requiring urgent surgical correction. We herein describe the diagnostic difficulties in a case of neonatal CTD, who developed increasingly severe and unresponsive cyanosis, first interpreted as postnatal maladjustment with pulmonary hypertension. The failure to respond to oxygen and pulmonary vasodilators led us to reconsider a different diagnosis. The use of contrast echocardiography improved the diagnostic performance of transthoracic echocardiogram (TTE) and revealed a massive right-to-left shunt secondary to the presence of an atrial membrane that required urgent surgery.
ABSTRACT
BACKGROUND: In COVID-19 patients, aldosterone via angiotensin-converting enzyme-2 deregulation may be responsible for systemic and pulmonary vasoconstriction, inflammation, and oxidative organ damage. AIM: To verify retrospectively the impact of the mineralcorticoid receptor antagonist canrenone i.v. on the need of invasive ventilatory support and/or all-cause in-hospital mortality. METHODS: Sixty-nine consecutive COVID-19 patients, hospitalized for moderate to severe respiratory failure at Fondazione Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Ospedale Maggiore Policlinico of Milan, received two different therapeutic approaches in usual care according to the personal skills and pharmacological management experience of the referral medical team. Group A (n = 39) were given vasodilator agents or renin-angiotensin-aldosterone system (RAAS) inhibitors and group B (n = 30) were given canrenone i.v. RESULTS: Among the 69 consecutive COVID-19 patients, those not receiving canrenone i.v. (group A) had an event-free rate of 51% and a survival rate of 64%. Group B (given a mean dose of 200 mg/q.d. of canrenone for at least two days of continuous administration) showed an event-free rate of 80% with a survival rate of 87%. Kaplan-Meier analysis for composite outcomes and mortality showed log rank statistics of 0.0004 and 0.0052, respectively. CONCLUSIONS: The novelty of our observation relies on the independent positive impact of canrenone on the all-cause mortality and clinical improvement of COVID-19 patients ranging from moderate to severe diseases.