A 33-year-old woman with aortic valve stenosis status-post Ross at age 6 years developed symptomatic right heart failure from right ventricle to pulmonary artery conduit stenosis. Conduit rehabilitation and transcatheter pulmonary valve replacement resulted in acute left atrial hypertension and respiratory failure requiring venoarterial extracorporeal membrane oxygenation and atrial septal defect creation as a bridge to recovery.
View Article and Find Full Text PDFBackground: Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of life. Patients with Beta-;TM may develop cardiac iron toxicity secondary to transfusion dependence. Cardiovascular magnetic resonance (CMR) T2*, a technique designed to quantify myocardial iron deposition, is a driving component of disease management.
View Article and Find Full Text PDFTransfusional iron overload represents a substantial challenge in the management of patients with sickle cell disease (SCD) who receive chronic or episodic red blood cell transfusions. Iron-induced cardiomyopathy is a leading cause of death in other chronically transfused populations but rarely seen in SCD. Study objectives were to: (i) examine the extent of myocardial and hepatic siderosis using magnetic resonance imaging (MRI) in chronically transfused SCD patients, and (ii) evaluate the relationship between long-term (over the 5 years prior to enrolment) mean serum ferritin (MSF), spot-ferritin values and liver iron content (LIC) measured using MRI and liver biopsy.
View Article and Find Full Text PDFBackground: Palliative procedures performed before the Fontan procedure may require ligation of the subclavian arteries, thereby affecting flow to the vertebral arteries. In adults with functionally univentricular heart disease, the implications of altered brainstem vascular anatomy for perioperative management of failing Fontan circulation are not known.
Methods: We identified abnormal posterior circulation anatomy in an adult patient with failing Fontan circulation who experienced a brainstem stroke after Fontan conversion.
Objective: The objective of our study was to determine the adverse reaction rate associated with the administration of blood pool contrast material in children and young adults.
Materials And Methods: A review of the MRI and pharmacy databases identified all patients who received gadofosveset trisodium from October 1, 2011, to June 30, 2014. Patients were classified as having been anesthetized or not anesthetized for the MRI examinations.
We present an analysis of 3D blood flow in two cases of Sinus of Valsalva to right heart fistulae based on 4D flow MRI. Despite similar underlying pathology, 3D visualization revealed intricate differences in flow patterns connecting the systemic and pulmonary circulation. The cases illustrates the potential of 4D flow MRI to complement the evaluation of complex structural heart disease by assessing complex flow dynamics and providing quantitative information of flow ratios and flow rates.
View Article and Find Full Text PDFBackground: The adult practice for ECG-gated single-source 64-slice coronary CTA (CCTA) includes administering beta-blockers to reduce heart rate. There are limited data on this process in children.
Objective: To evaluate the safety and efficacy of a drug regimen to decrease heart rate before performing CCTA in children.
Background: The evolving operative strategy and course of 111 consecutive Fontan conversions with arrhythmia surgery and pacemaker therapy were reviewed to identify risk factors for poor outcome.
Methods: Since 1994, 111 patients (mean age 22.5 +/- 7.
Purpose: To determine normative R2* values in the liver and heart at 3T, and establish the relationship between R2* at 3T and 1.5T over a range of tissue iron concentrations.
Materials And Methods: A total of 20 healthy control subjects and 14 transfusion-dependent patients were scanned at 1.