Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction.

OBJECTIVES: To investigate diagnostic routes, echocardiographic substrates, outcomes and prognostic factors in patients with isolated ventricular non-compaction (IVNC) identified by echocardiographic laboratories with referral from specialists and primary care physicians. PATIENTS AND DESIGN: Since 1991, all patients with suspected IVNC were flagged and followed up on dedicated databases. Patients were divided into symptom-based and non-symptom-based diagnostic subgroups. RESULTS: 65 eligible patients were followed up for 6-193 months (mean 46 (SD 44). In 53 (82%) patients, IVNC was associated with variable degrees of left ventricular (LV) dilatation and hypokinesia, and in the remaining 12 (18%) LV volumes were normal. Diagnosis was symptom based in 48 (74%) and non-symptom based in 17 (26%) (familial referral in 10). The non-symptom-based subgroup was characterised by younger age, lower prevalence of ECG abnormalities, better systolic function and lower left atrial size, whereas the extent of non-compaction was not different. No major cardiovascular events occurred in the non-symptom-based group, whereas 15 of 48 (31%) symptomatically diagnosed patients experienced cardiovascular death or heart transplantation (p = 0.01, Kaplan-Meier analysis). Independent predictors of cardiovascular death or heart transplantation were New York Heart Association class III-IV, sustained ventricular arrhythmias and left atrial size. CONCLUSIONS: IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental or familial discovery of IVNC have an encouraging outlook, whereas those who have symptoms of heart failure, a history of sustained ventricular tachycardia or an enlarged left atrium have an unstable course and more severe prognosis.

Current indications for contrast echocardiography imaging.

UNLABELLED: The assessment of regional ventricular function is dependent on good endocardial definition. Suboptimal images can be converted to diagnostic recordings in the majority of patients by contrast agents, which have become an indispensable aid in rest and stress echocardiography. In particular for stress echocardiography image quality is essential and contrast administration is of great importance. However this diagnostic procedure must be performed following the indications which reflect the risks of the procedure and consider the benefits of an accurate diagnosis on further patient management. The contraindications recently introduced in the use of the echo-contrast agent SonoVue for acute cardiac patients reflect the same contraindications which have been applied in stress echocardiography for several years. Clinical trials and post-marketing surveillance have demonstrated that this approach is safe with no fatalities reported. For all ultrasound contrast media, side effects have been reported but they are usually mild. However, rare allergic and potentially life threatening reactions may occur and the investigators have to be prepared for such an event with appropriate drugs stored in the echo department. In a recent retrospective analysis in 751 consecutive stress echocardiograms the use of contrast during dobutamine stress echocardiography was not associated with an increased risk of side effects. The incidence of side effects was very low and different in patients receiving Optison, SonoVue or without contrast agent. CONCLUSION: Ultrasound contrast agents are licensed for improvement of endocardial border definition. Data from clinical trials and wide clinical experience indicate an excellent risk/benefit ratio if the current contraindications are applied.