Cardiac arrest secondary to arrhythmogenic right ventricular cardiomyopathy in an adolescent male.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, genetically-inherited cardiomyopathy that may be fatal. We present the case of a 17 year old male who presented after a witnessed cardiac arrest with indeterminate echocardiogram and electrocardiogram (ECG) findings for a specific etiology. Genetic testing revealed a mutation in the PKP2 and DSC2 genes, consistent with ARVC. This report outlines the presentation of ARVC as an aborted sudden cardiac death episode in a previously asymptomatic teenager, investigations for ARVC and highlights the importance of adequate cardiopulmonary resuscitation in the overall prognosis. Implantable cardiac defibrillator (ICD) placement for secondary prevention is necessary.

Sudden cardiac arrest as a presenting symptom of mitral valve prolapse.

Mitral valve prolapse (MVP) is a common and predominantly benign condition; however, occasional reports of cardiac arrest in individuals with MVP have raised concern for a malignant subtype. The risk of sudden cardiac arrest in MVP is twice as high as that in the general population. The exact aetiology and risk predictors are elusive, but identification is necessary, if we are to protect patients at risk for cardiac arrest. This report highlights cardiac arrest as the initial presenting symptom of MVP. Rapid initiation of bystander cardiopulmonary resuscitation and time to first shock are key predictors of prognosis. Better screening to identify individuals with malignant phenotypes may aid in reducing the morbidity and mortality in patients with a predisposition for life-threatening arrhythmias.

Rare Etiology of Acute Embolic Stroke in a 17-Year-Old Girl: Cardiac Papillary Fibroelastoma.

A cardiac papillary fibroelastoma is a benign, pedunculated tumor with strand-like projections arising from the endocardium. While often discovered incidentally, these tumors can embolize resulting in major neurologic events. There is a dearth of pediatric literature describing the necessity and optimal timing of surgical intervention for this rare primary cardiac tumor after a cerebrovascular event. We report an interesting case and the subsequent management dilemma in a teenage patient presenting with acute embolic strokes secondary to an aortic valve papillary fibroelastoma. Despite its rarity, a high index of suspicion should be maintained for such tumors in previously healthy children presenting with acute stroke.

Endobronchial schwannoma presenting with bronchial obstruction.

Schwannomas are relatively uncommon, benign nerve sheath tumors. Thoracic schwannomas most often appear in the posterior mediastinum. Pulmonary schwannomas are exceedingly rare and can present a diagnostic challenge. We present a case of an endobronchial schwannoma presenting with bronchial obstruction and review the literature of this unusual entity.

Lead astray: minimally invasive removal of a pacing lead in the left ventricle.

Pacemaker and implantable cardioverter defibrillator (ICD) leads are placed routinely with few notable complications. A rarely described complication of transvenous lead placement is malpositioning into the left ventricle. This situation can cause additional complications in the form of thromboembolic events. We present a case of a malpositioned left ventricular lead that was successfully removed with a minimally invasive technique.

Survival benefit of endovascular descending thoracic aortic repair for the high-risk patient.

BACKGROUND: Despite acceptable results reported with endovascular thoracic aortic repair (TEVAR), recent studies have questioned the merit of repair in asymptomatic patients considered high risk for open surgery. In this group, advanced age or comorbid conditions may reduce life expectancy, thus limiting the benefit of elective aneurysmectomy. This study was conducted to determine whether elective TEVAR improves survival for this cohort. METHODS: Forty-six asymptomatic patients with descending thoracic aortic disease were considered high risk for open surgery for reasons of age of 80 years or older (47.8%) or comorbid conditions (84.8%), and were subsequently evaluated for elective TEVAR. Of these, 21 underwent TEVAR, while another 25 patients were excluded from TEVAR on the basis of unfavorable anatomy or refused intervention. RESULTS: The mean age of the cohort was 77.0 +/- 7.0 years (p = 0.9 between groups). Prevalent comorbid conditions were similar between groups, and included coronary artery disease (p = 1.0), chronic obstructive pulmonary disease (p = 1.0), and peripheral vascular disease (p = 0.23). Mean maximum aortic diameter was 6.0 +/- 1.4 cm (p = 0.54 between groups). Indications for intervention included fusiform aneurysm (65.2%) and pseudoaneurysm or penetrating ulcer (32.6%). No 30-day mortality was observed after TEVAR. All-cause mortality in the entire cohort was 50%. Median actual time to mortality was different between groups (control, 9.2 months versus TEVAR, 24.9 months; p = 0.01). Life-table analysis demonstrated improved survival for TEVAR at 24 months (p = 0.05). CONCLUSIONS: Although the overall prognosis for the asymptomatic patient with descending thoracic aortic disease at high risk for open surgery is poor, elective endovascular repair improves survival and should be considered a therapeutic option in this setting.

Resection of the descending thoracic aorta: outcomes after use of hypothermic circulatory arrest.

BACKGROUND: Use of hypothermic circulatory arrest (HCA) for operations on the descending thoracic aorta is controversial. While deep hypothermia may provide better end-organ and spinal cord function, prolonged cardiopulmonary bypass and circulatory arrest may increase morbidity. This study assessed outcomes after use of HCA for descending thoracic aortic resection in a large cohort of consecutive patients. METHODS: Hypothermic circulatory arrest was utilized if arch or extensive descending thoracic aortic resection was required, or if aortic pathology precluded cross-clamping. One hundred thirty-two patients (mean age, 61.3 years) were identified. Diagnosis included fusiform (41.2%) or saccular aneurysm (10.7%) and acute (4.6%) or chronic (38.9%) dissection. Twenty-one patients presented with rupture. Arch resection (distal arch 100, total arch 11) was required in 111 patients (84.1%). The extent of descending thoracic aortic resection (required in 94%) included proximal third in 41 patients, proximal two-thirds in 6, and complete thoracic aorta in 77. The proximal anastomosis was performed with total body HCA while the distal anastomosis was constructed with lower body HCA only (duration upper body HCA 33.7 +/- 8.0 minutes; total duration lower body HCA 71.3 +/- 24.2 minutes). RESULTS: Thirty-day mortality was 6.0%. Neurologic events included stroke (6.8%) and permanent lower extremity paralysis-paresis (4.5%). Temporary dialysis was needed in 7 (5.3%), though only 2 patients required permanent dialysis (1.9%). Independent predictors of a composite endpoint of death, stroke, permanent paralysis, or dialysis included duration of lower body HCA (p = 0.03) and major postoperative infection (p = 0.003). CONCLUSIONS: Adjunctive use of deep hypothermic circulatory arrest for descending thoracic aortic resection affords excellent preservation of end-organ and spinal cord function with acceptable rates of mortality and significant morbidity.

Long-term results from a 12-year experience with endovascular therapy for thoracic aortic disease.

BACKGROUND: Endovascular approaches promise to revolutionize therapy for thoracic aortic disease. This study describes a long-term analysis of endovascular thoracic aortic repair. METHODS: Seventy-three patients (mean age, 67.4 years) underwent endovascular thoracic aortic repair from 1993 to 2005. Indications for intervention included aneurysm (38%), dissection (23%), or penetrating ulcer or pseudoaneurysm (34%). Rupture was present in 16 patients (22%). Seventy-one percent were considered high risk for open surgery for reasons of age or comorbid conditions. Treated segments included ascending aorta (n = 1), distal arch (n = 24), and proximal (n = 50) or distal (n = 55) descending aorta. The total descending thoracic aorta was covered in 31 patients. Procedural success was achieved in 96%. Devices were delivered by femoral (79%), retroperitoneal iliac (18%), or carotid (2.7%) exposure. Devices used included Excluder (n = 30), Talent (n = 23), Zenith (n = 3), AneuRx (n = 5), and custom-fabricated (n = 14). Follow-up was 100% complete. RESULTS: Thirty-day mortality was 5.5%. Significant morbidity included stroke (8.2%) and need for dialysis (4.1%). Although 3 patients had transient spinal cord ischemia (4.1%), none had permanent sequelae. Intervention for fusiform aneurysm was independently associated with a composite end point of 30-day mortality, need for dialysis, and stroke (p = 0.015). Eight patients (11%) had new or persistent endoleaks, and aortic reintervention was performed in 7 patients (9.6%). Mean survival for the entire cohort was 46.8 +/- 5.1 months. Intervention for penetrating ulcer or pseudoaneurysm (p = 0.045) was independently associated with long-term all-cause mortality. CONCLUSIONS: An endovascular approach produces acceptable results for a broad range of thoracic aortic disease. However, the potential for endoleak or need for reintervention mandates continued close follow-up to achieve satisfactory long-term results.