Spontaneous regression of a large rhabdomyoma of the interventricular septum.

We report the case of a large congenital rhabdomyoma of the interventricular septum diagnosed prenatally. The foetus was strictly monitored with ultrasound throughout the gestation period showing that the mass had increased in size until delivery. Despite the size of the mass, which appeared to occupy the right ventricle, the baby presented no symptoms both in utero and after birth. Serial echocardiography was used to document the regression of the mass in childhood.

A modified echocardiographic approach improves reliability of superior vena caval flow quantification.

OBJECTIVE: To assess accuracy and repeatability of a modified echocardiographic approach to quantify superior vena cava (SVC) flow volume that uses a short-axis view to directly measure SVC area and a suprasternal view to measure flow velocity, both at the level of the right pulmonary artery. SETTING: Three tertiary-level neonatal intensive care units. DESIGN: This was a multicentre, prospective, observational study. Accuracy of the traditional and modified approach was first assessed by comparing echo measurements according to both techniques with Phase contrast MRI (PCMRI) assessments, in a cohort of 10 neonates. In a second cohort of 40 neonates, intraobserver scan-rescan repeatability and interobserver analysis-reanalysis repeatability were assessed by repeated SVC flow echo measurements, according to both techniques. RESULTS: The traditional echocardiographic approach to assessment of SVC flow had a moderate agreement with PCMRI (r2 0.259), a scan-rescan intraobserver repeatability index (RI) of 37% (limits of agreement (LOA) -47/+51 mL/kg/min) and an interobserver analysis-reanalysis RI of 31% (LOA -38/+40 mL/kg/min). The modified approach showed a stronger agreement with PCMRI (r2 0.775), an improved intraobserver scan-rescan repeatability (RI 22%, LOA -24/+18 mL/kg/min) and improved interobserver analysis-reanalysis repeatability (RI 18%, LOA -18/+20 mL/kg/min). CONCLUSIONS: Echocardiographic assessment of SVC flow volume by tracing area from a short-axis view and measuring velocity-time integral from a suprasternal view offered an improvement in accuracy and repeatability, building on the traditional approach previously described.

Hemodynamic predictors of long term survival in end stage cystic fibrosis.

BACKGROUND: Pulmonary hypertension (PH) is often found in cystic fibrosis (CF) patients affected by end-stage lung disease but its impact on outcome remains unclear. Pulmonary arterial compliance (PAC) is an important determinant of right ventricle (RV) workload and it is a strong predictor of survival in other forms of PH. The aim of this study is to investigate whether PAC is a predictor of long-term prognosis in a population of CF patients affected by advanced lung disease. METHODS: Between 2000 and 2014, 178 patients with CF have been evaluated for lung transplantation in our CF Center. Right heart catheterization (RHC) and follow up data were retrievable and analyzed in 141 of them. PAC was defined as the ratio between stroke volume (SV) and pulse pressure (PP) at heart catheterization. The association of PAC with survival was tested at 4 years and compared to other hemodynamic parameters. RESULTS: PH prevalence was 56.4%. Most patients had mild elevation of pulmonary artery pressure (PAP). No difference in mortality was observed in patients with PH compared to patients with normal PAP (HR 0.95: 95% CI 0.49-1.89, p=0.89). At receiver operating characteristic curve (ROC) analysis, the optimal prognostic cut-off point of PAC was 1.95 ml/mmHg. An impaired PAC (≤1.95 ml/mmHg) was a strong independent predictor of long-term mortality (HR 3.44: 95% CI 1.51-7.85: p=0.003). CONCLUSIONS: Impaired PAC is associated with poor prognosis in CF patients awaiting lung transplantation. Other traditional hemodynamic parameters add no prognostic information.

Uncorrected Ebstein's anomaly with atrial septal defect complicated by brain abscess in an adult patient.

: We report the case of a 56-year-old male patient affected by a severe form of Ebstein's anomaly (type C of Carpentier classification) with secundum atrial septal defect, who presented to the emergency department with impaired consciousness, seizures, and trismus. The brain computed tomography scan showed evidence of a mass located in the frontal lobe, confirmed by brain MRI consistent with brain abscess. Both echocardiography and cardiac MRI showed no evidence of valvular vegetation. This case shows how the combination of increased atrial pressure and bidirectional shunt through atrial septal defect may lead to paradoxical embolization.

Long-term independent predictors of positive response to cardiac resynchronization therapy.

AIMS: Cardiac resynchronization therapy (CRT) is currently considered an important breakthrough in the treatment of selected patients with refractory heart failure. However, long-term predictors of mortality, morbidity and time to recovery of ventricular function for those patients who respond positively to CRT remain poorly investigated. METHODS: This is a retrospective follow-up study involving one hospital. Between August 2004 and October 2008, 211 consecutive patients with refractory heart failure received a CRT device in the Cardiology Division of Ospedale Civile Maggiore in Verona. The clinical characteristics studied were age, sex, heart rhythm, left ventricular end-systolic volume/body surface area (LVESV/BSA), left ventricular ejection fraction, QRS duration, type of bundle-branch block, cause, New York Heart Failure Association functional class, pharmacological therapy and lead position. The objective of this study was to evaluate the effect of several baseline characteristics on long-term prognosis in heart failure patients treated with CRT. RESULTS: Nonischemic cause, left bundle-branch block and a basal LVESV/BSA of 106 ml/m or less were the only independent predictors of a positive response to CRT (P < 0.005). Additionally, a reduction in LVESV/BSA after CRT was associated both with increased survival and reduced rehospitalization for heart failure (P < 0.005). CONCLUSION: A better selection of patients on the basis of cause, type of bundle-branch block and basal LVESV/BSA can increase the number of patients that would benefit from CRT.