Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination.

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.

Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.

Pseudoaneurysm of the right ventricle outflow tract (RVOT) is a rare complication in pediatric cardiac surgery. We report a patient who developed a right ventricular pseudoaneurysm 8 months after RVOT enlargement using a pericardial patch for infundibular pulmonary stenosis. Our patient was born with severe pulmonary valvular stenosis and treated with percutaneous balloon valvotomy in the neonatal period. Six months later, she developed infundibular pulmonary stenosis, which required surgical resection of right ventricle infundibular trabeculations and bovine pericardial patch enlargement. The postoperative period was normal. She was readmitted to hospital 5 months later complaining of wheezing, coughing and shortness of breath. Echocardiography showed a huge aneurysmal dilatation of the outflow patch in connection with the right ventricular cavity. The patient underwent resection of the pseudoaneurysm and former patch, followed by interposition of a bovine jugular vein conduit between the RVOT and pulmonary bifurcation. The early postoperative period was uncomplicated. On echocardiography, no significant residual gradient was measured through the conduit and there was no insufficiency of the valve. RVOT reconstruction with patch enlargement, homograft or conduit implantation can be the origin of pseudoaneurysms. Although their incidence is rare, they are often asymptomatic before becoming quite large and causing compression symptoms as in our patient with respiratory complaints due to airway compression. It is important to follow up these patients closely, especially in the first year after surgery since most aneurysms develop within 6 months of surgery.

Biodegradable mitral annuloplasty for congenital ischemic mitral regurgitation.

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is associated with ischemic mitral regurgitation through a combination of papillary muscle ischemia or permanent fibrosis, left ventricular free wall ischemic dyskinesis and left ventricular dilatation. We report the successful management of a 27-month-old girl with ALCAPA and severe mitral regurgitation using a biodegradable mitral annuloplasty ring. It could represent a novel tool for mitral valve repair in ALCAPA-associated ischemic mitral regurgitation.

Amyand's hernia: 10 years' experience.

BACKGROUND: Amyand's hernia is an atypical groin hernia which contains the vermiform appendix. The aim of this study was to review a single institution's experience in the clinical presentation, management and prognostic factors of this rare hernia. METHODS: The authors reviewed records of all patients undergoing hernia surgery from 1996 to 2006 at their institution, a tertiary care, University-affiliated hospital. RESULTS: Twelve patients (six men) with a median age of 88 years (range 60-97) were included. Six presented with right inguinal hernias and six presented with right femoral hernias. All required emergency surgery: eight for strangulated hernias, two for hernias with lower quadrant peritonism and two for incarcerated hernia. Despite small differences in outcome and length of hospitalisation between Amyand types, appendix inflammation, pre-operative blood examinations and hernia localisation, only right lower quadrant peritonism as a presenting sign (p=0.004) and age greater than 90 years old (p=0.04) were significantly associated with a poor outcome. CONCLUSION: Amyand's hernia is a rare hernia which is seldom diagnosed before operation. It must be considered in the evaluation of a strangulated or incarcerated hernia. Further studies are required to define the optimal surgical strategy, prognostic factors and risks of hernia recurrence.

Mitral tendon prolapsing into the left ventricular outflow tract.

Intracardiac masses of the mitral valve are rare. Their differential diagnosis is wide, ranging from tumors (myxomas, lipomas and fibroelastomas), thrombi and abnormal muscular or fibrous bands. We report a case and management. A 68 year-old asymptomatic female who had undergone coronary angioplasty and stent placement in the left anterior descending artery for acute myocardial infarction four years earlier, was shown to have, on routine follow-up, an intracardiac mass originating from the anterior leaflet of the mitral valve and prolapsing into the left ventricular outflow tract (LVOT). The patient underwent surgical excision of the mass under cardiopulmonary bypass, to prevent cerebral or coronary embolization and sudden death due to the highly sensitive location of the mass, in the high-velocity flow LVOT. A transverse aortotomy provided exposure of the ventricular surface of the anterior mitral leaflet and revealed a fusiform mass attached to the medial segment of the anterior leaflet, resembling a secondary cordae, measuring 20 by 3 mm. The implantation was calcified on the ventricular aspect of the anterior mitral leaflet. This mass was completely excised. Postoperative recovery was uneventful. Peroperative and postoperative transesophageal echocardiography were normal. Histological examination showed a partially necrosed and calcified fibrous tissue lined by endothelium. The final diagnosis was that of a mitral tendon. Intracardiac masses of the mitral valve are rare lesions, mostly papillary fibroelastomas and myxomas and more rarely mitral tendons, which require surgical resection for prevention of embolization. The definitive diagnosis is often only obtained on histological analysis.

Valve repair using biodegradable ring annuloplasty: from bench to long-term clinical results.

  Annulus remodeling and stabilization with a ring is a necessary step in mitral and tricuspid valve repair to maintain effective leaflet coaptation and improve long-term results. Although conventional rings meet the basic needs of adults, they do not preserve the changes in shape and size occurring during the cardiac cycle, and do not allow growth of the native annulus in children. The bioring annuloplasty ring was developed to allow for annular stabilization, while remaining biodegradable and allowing for growth. It is a curved "C" segment of poly-1,4-dioxanone polymer located on a non-degradable polyvinyl monofilament suture equipped with a stainless steel needle at each extremity. This ring is inserted subendocardially directly into the mitral or tricuspid annulus, away from blood contact. Animal model experiments have shown that it degrades within 12 months of implantation and is replaced by fibrous tissue, which stabilizes the annulus durably, while allowing for annular growth in children. We review the published data, from bench to bedside, as well as the early, mid and long-term clinical outcomes using the biodegradable ring, which shows that biodegradable rings remodel the annulus, reinforce the repair, restore the function of the atrioventricular valve and maintain the three dimensional dynamic motion and geometry of the mitral and tricuspid valves annulus. Growth potential is preserved in children. The mid- and long-term results showed that degradation of the device occurred without negative observable consequences.

Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.

The left aortic arch with an aberrant right subclavian artery, or arteria lusoria, is the most common aortic arch anomaly, occuring in 0.5-2.5% of individuals. Four vessels arise sequentially from the aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery and the aberrant right subclavian artery, which crosses upwards and to the right in the posterior mediastinum. It results from a disruption in the complex remodelling of the paired branchial arches, typically of the right dorsal aorta distal to the sixth cervical intersegmental artery. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. It is usually asymptomatic. When symptomatic, it produces dysphagia lusoria or dyspnea and chronic coughing. Treatment is indicated for symptomatic relief of dysphagia lusoria and for prevention of complications due to aneurysmal dilatation.

Primary intrapulmonary thymoma: a systematic review.

AIM: This article reviews the literature on the clinical features, diagnosis and management of primary intrapulmonary thymoma. METHODS: Medline, Embase and Cochrane Library searches were performed on all relevant Anglo-Saxon language articles. The search words included "primary pulmonary thymoma" and "intrapulmonary thymoma". Secondary references were obtained from key articles. Prognostic and treatment strategies were analyzed by the Kaplan-Meier method, comparisons between curves were made using log rank test. RESULTS: The searches yielded 25 cases of primary intrapulmonary thymoma. Median follow-up was 9 months (1 day to 13 years). At follow-up, 14 patients were tumor free, one patient had a local recurrence 8 years after radiotherapy, one patient responded favorably to radiotherapy, six patients died and three patients were lost to follow-up. The presence of a paraneoplastic syndrome decreased survival (P=0.02), however, histological subgroup (P=0.216), clinical stage (P=0.63) and tumor size (P=0.288) did not affect survival. Survival in surgically managed patients was significantly better than in conservatively managed patients (P=0.039). Adjuvant radiotherapy did not provide any benefit (P=0.4). CONCLUSION: Complete resection of primary intrapulmonary thymomas appears sufficient in non-malignant tumors. Because of the risk of late local recurrence, long-term regular clinical follow-up is warranted.