Correlation between non-invasive to invasive right-heart data in paediatric heart transplant patients.

BACKGROUND: Paediatric studies have shown serum N-terminal pro b-type natriuretic peptide levels to be a valuable tool in the surveillance of myocardial function and an early biomarker for rejection in transplant patients. The correlation between low mean right atrial pressure and increased inferior vena cava collapsibility index is well studied in adults. Our study aims to assess correlation between non-invasive measurements (serum N-terminal pro b-type natriuretic peptide, inferior vena cava dimensions collapsibility, tricuspid regurgitation, and left ventricular remodelling index to invasive mean right atrial pressure in paediatric heart transplant patients). METHODS: A single centre, retrospective chart review of the paediatric transplant patients from 0 to 21 years of age was performed between 2015 and 2017. Thirty-nine patients had complete data which includes cardiac catheterisation, transthoracic echocardiogram, and serum N-terminal pro b-type natriuretic peptide levels done within a two weeks of interval. RESULTS: A higher inferior vena cava collapsibility index correlated with a lower mean right atrial pressure (r = -0.21, p = 0.04) and a larger inferior vena cava diameter in expiration indexed to body surface area (IVCmax/BSA0.5) correlated with a higher mean right atrial pressure (r = 0.29, p = 0.01). There was a correlation between elevated N-terminal pro b-type natriuretic peptide and inferior vena cava collapsibility index (r = -0.38, p = 0.0001), IVCmax/BSA0.5 (r = 0.25, p = 0.0002), and mean right atrial pressure (r = 0.6, p = 0.0001). CONCLUSION: Serum N-terminal pro b-type natriuretic peptide levels correlated to non-invasive measurements (inferior vena cava collapsibility index and IVCmax/BSA0.5) and to the invasive mean right atrial pressure. Non-invasive (IVC-CI IVCmax/BSA0.5) correlates with elevated mean right atrial pressure in this population. Together, these may serve as a reliable surveillance tool in assessing right heart filling pressures and cardiac function within the paediatric heart transplant patient.

Sinus node dysfunction masquerading as complete atrioventricular block in the setting of atrial parasystole after heart transplantation.

Heart transplantation is the gold standard for treatment of end stage heart failure. Atrial parasystole (AP), defined as the presence of an additional dissociated atrial pacemaker on a surface electrocardiogram, has been described to be seen after heart transplantation using biatrial surgical technique. AP may pose a challenge in diagnosing underlying rhythm disturbances in these patients. Here, we report a rare phenomenon of junctional escape rhythm with sinus node dysfunction, mimicking as complete atrioventricular block due to the presence of an AP, in a heart transplant recipient.

Infantile Onset Hypertrophic Cardiomyopathy Secondary to PRKAG2 Gene Mutation is Associated with Poor Prognosis.

Hypertrophic cardiomyopathy (HCM) is the second most prevalent form of cardiomyopathy in children. The etiology of the HCM is heterogeneous, so is the age of onset of symptoms. The HCM associated with metabolic disorders and genetic syndromes presents early in childhood. There are very few case reports of early-onset infantile HCM secondary to the PRKAG2 gene. Here, we report a case of HCM in a neonate diagnosed prenatally and eventually diagnosed with a missense mutation in the PRKAG2 gene.

Incidence and factors influencing the spontaneous closure of Fontan fenestration.

INTRODUCTION: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long-term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration. METHODS: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at our center. Preoperative, intraoperative and postoperative variables including the status of the Fontan fenestration were extracted and analyzed. RESULTS: Of 67 patients included in the study, 15 (22%) had spontaneous closure of the fenestration. Of the remaining 52 patients, 11 (20%) had procedural closure of this fenestration (10 via cardiac catheterization and 1 via surgery) at a median duration of 3 months after the Fontan operation. Patients with higher preoperative pulmonary vascular resistance and a history of postoperative systemic venous thromboembolism had higher likelihood of having persistence of the fenestration with P value of .045 and .037, respectively. CONCLUSIONS: The rate of spontaneous closure of the Fontan fenestration was 22% in our study. Elevated preoperative pulmonary vascular resistance and history of systemic venous thromboembolism are predictive of persistent Fontan fenestration.