Noncompaction syndrome of the myocardium: pathophysiology and imaging pearls.

Patients with noncompaction syndrome of the myocardium may present with cardiac arrhythmia, thromboembolic events, or left ventricular failure. Associations with other diseases, including neurologic or other cardiac syndromes, have been noted. Noncompaction syndrome of the myocardium is primarily diagnosed by echocardiography; however, CT scanning and magnetic resonance imaging are useful tools for determining severity and the patient's prognosis. With these methods, high-resolution images of the myocardium are obtained that enable better recognition of the areas over which the trabeculae are distributed. Early diagnosis can improve the patient's survival by premature heart transplantation or implantation of a defibrillator. Also, as there are many reports of occurrence of this syndrome in several members of the same family (mainly X-linked inheritance), upon accurate and early diagnosis, the patient's family can be further screened.

Systemic to pulmonary venous shunt: imaging findings and clinical presentations.

Multislice computed tomography technology has enabled sophisticated insights into the evaluation of collateral venous pathways in the thorax. A small but well-established body of literature has described the multiple venous pathways in patients with central venous obstruction. In unusual circumstances, however, physiologically maladaptive pathways have been described involving systemic to pulmonary venous shunts. This paper describes 21 patients with systemic to pulmonary venous shunts having a wide spectrum of etiologies. Clinical presentations typically include cardiopulmonary symptomatology and paradoxical emboli. Ultrafast magnetic resonance and multislice computed tomography established the diagnosis of this uncommon but important entity.

Preoperative palliation of newborn obstructed total anomalous pulmonary venous connection by endovascular stent placement.

Patients with obstructed total anomalous pulmonary venous connection (TAPVC) usually present critically ill and continue to be extremely challenging with presurgical stabilization. We present an extra corporeal membrane oxygenation (ECMO)-dependent neonate with obstructed TAPVC that was successfully palliated with transvenous stent placement in the obstructed vertical vein.

Intra-atrial tissue plasminogen activator infusion for prosthetic valve thrombosis.

Thrombosis is a well-known and life-threatening complication of prosthetic mechanical valves. Therapy typically requires medical thrombolysis or surgical thrombectomy/valve replacement. We report the case of a thrombosed atrioventricular valve in a young boy with complex congenital heart disease, which was successfully treated with direct intra-atrial infusion of recombinant tissue plasminogen activator after failed attempts at systemic therapy. We present this treatment as an alternative to conventional medical therapy.

Evaluation of right ventricular fibrosis in adult congenital heart disease using gadolinium-enhanced magnetic resonance imaging: initial experience in patients with right ventricular loading conditions.

OBJECTIVE: Gadolinium-enhanced cardiac magnetic resonance imaging has been used to show myocardial fibrosis, a finding that appears as late gadolinium enhancement. Its role in the evaluation of right ventricular fibrosis in congenital heart disease is unclear. The purpose of this study was to demonstrate late gadolinium enhancement of the right ventricle in adult and adolescent congenital heart disease and to investigate the relationship between this enhancement and clinical and pathophysiological data. DESIGN: In total, 24 patients, 16 patients with congenital heart disease and right ventricular loading conditions and 8 controls, underwent gadolinium-enhanced viability imaging. Diagnoses varied and included repaired, palliated, and unrepaired lesions. The presence and extent of right ventricular late gadolinium enhancement was compared with patient clinical and hemodynamic data. Exact Wilcoxon tests, Fisher's exact tests, and Spearman's rank correlation were used to compare variables. RESULTS: Nine of 16 patients (56%) were found to have right ventricular late gadolinium enhancement, ranging from 5% to 80% of right ventricular myocardium affected (mean 36.1%, SD 29.7). The combination of right ventricular systolic pressure >or=98 mm Hg and systemic oxygen saturation

Perfusion abnormalities in congenital and neoplastic pulmonary disease: comparison of MR perfusion and multislice CT imaging.

The aim of this work was to assess magnetic resonance (MR) perfusion patterns of chronic, non-embolic pulmonary diseases of congenital and neoplastic origin and to compare the findings with results obtained with pulmonary, contrast-enhanced multislice computed tomography (CT) imaging to prove that congenital and neoplastic pulmonary conditions require MR imaging over the pulmonary perfusion cycle to successfully and directly detect changes in lung perfusion patterns. Twenty-five patients underwent concurrent CT and MR evaluation of chronic pulmonary diseases of congenital (n=15) or neoplastic (n=10) origin. Analysis of MR perfusion and contrast-enhanced CT datasets was realized by defining pulmonary and vascular regions of interest in corresponding positions. MR perfusion calculated time-to-peak enhancement, maximal enhancement and the area under the perfusion curve. CT datasets provided pulmonary signal-to-noise ratio measurements. Vessel center-lines of bronchial arteries were determined. Underlying perfusion type, such as pulmonary arterial or systemic arterial supply, as well as regions with significant variations in perfusion were determined statistically. Analysis of the pulmonary perfusion pattern detected pulmonary arterial supply in 19 patients; six patients showed systemic arterial supply. In pulmonary arterial perfusion, MR and multislice CT imaging consistently detected the perfusion type and regions with altered perfusion patterns. In bronchial arterial supply, MR perfusion and CT imaging showed significant perfusion differences. Patients with bronchial arterial supply had bronchial arteries ranging from 2.0 to 3.6 mm compared with submillimeter diameters in pulmonary arterial perfusion. Dynamic MR imaging of congenital and neoplastic pulmonary conditions allowed characterization of the pulmonary perfusion type. CT imaging suggested the presence of systemic arterial perfusion by visualizing hypertrophied bronchial arteries.

Endovascular stent implantation in patients with stenotic aortoarteriopathies: early and medium-term results.

Data regarding stent implantation for stenotic aortoarteriopathy (SAA) are incomplete. We report on nine patients with this rare syndrome who underwent arterial stent implantation. Indications, results, and complications for patients with SAA were reviewed. Nine patients underwent 11 procedures. Twenty-two stents were implanted in the aorta or brachiocephalic vessels. Five patients had diffuse stenoses, three patients had middle aortic syndrome, and one patient had thoracic and abdominal coarctation. Associated diagnoses included Williams syndrome (2), neurofibromatosis (2), Takayasu's (1), and congenital rubella (1). Median gradient was 60 mm Hg (20-140 mm Hg). Poststent gradient was 15 mm Hg (0-60 mm Hg; P < 0.001). Additional stents were implanted in two patients and five underwent stent redilation. Two patients (22%) were found to have aneurysm formation. Stent implantation effectively provides gradient relief in SAA. Gradient reduction persists or is amenable to redilation. Importantly, however, uncomplicated stent implantation does not preclude aneurysm formation and may be more common than in traditional patient groups.