This paper reviews the evolution of surgical technique that has occurred with the Fontan procedure since it was first introduced more than 25 years ago. Although there has been recent enthusiasm at some centers for a return to Fontan's original concept of use of a conduit to achieve the Fontan pathway, we continue to believe that the lateral tunnel with double cavopulmonary anastomosis is the preferred approach. The late incidence of arrhythmias with the lateral tunnel at 10 years follow-up is remarkably low. On the other hand conduits present a risk of outgrowth and pseudointima accumulation. Even small gradients, e. g. less than 4mm, will be poorly tolerated over the longer term and may result in an increased incidence of cirrhosis and protein losing enteropathy.
Overall there has been a remarkable improvement in the early and late results of the Fontan procedure over the last decade. The role of the bi-directional Glenn shunt as either a staging procedure or definitive palliation when performed in conjunction with supplementary pulmonary blood flow needs to be defined by a prospective randomized study. Likewise the role of the fenestration also needs to be defined by a prospective randomized study including careful studies of late exercise capacity and maximal oxygen consumption. Another issue that needs to be defined by careful prospective randomized study is the importance of anti-coagulation with coumadin versus aspirin in reducing the incidence of thromboembolism.

Pulmonary arterionevous malformation (PAVMs) are a rare disorder with an incidence of 2-3/1,000,000 population. Approximately 70% of cases are associated with Osler-Weber-Rendu Disease. This intrapulmonary malformation causes hypoxemia and dyspnea largely attributed to the right to left shunting.

We present a case of 25-year-old male who was admitted for fever and headaches with chronic history of epistaxis, hemoptysis, cyanosis and clubbing. Central cyanocis and clubbing were evident with hypoxemia of 65%. Heart sounds and peripheral pulses were normal. There was no systolic bruit noted in the lung bases. Multiple telangiectasias were seen in the truncal area and abdomen. Chest x-ray revealed multi-chambered cardiomegaly with no opacifications. CBC showed erythrocytosis with hematocrits of 0.68-0.78. Transthoracic echocardiography showed intact interatrial and interventricular septum, with contrast study suggestive of intrapulmonary shunting. CT angiography revealed PAVMs in bilateral lung fields. Pulmonary angiography demonstrated diffuse PAVMS in the left lung with 3 large PAVMs with multiple feeders > 7mm and smaller PAVMs in the Right lower lobe. The diagnosis was Multiple Pulmonary Arteriovenous Malformation, Osler-Weber-Rendu Disease, Brain Abscess Left Temporoparietal area. Craniotomy with brain abscess evacuation was done. Left pneumonectomy or Right lobectomy was considered but was deemed unacceptable due to high morbidity and mortality. Endovascular coil embolization was done on the 3 large fistulas on the Left lobe. Post procedure, his arterial oxygenation improved to 96%. He has no recurrence of hemoptysis, no headaches and with less episodes of shortness of breath 2 months on follow-up. Diagnosing the complex diffuse intrapulmonary malformations requires a high index of suspicion among patients with chronic cyanosis, chronic history of bleeding, and brain abscess. Treatment of this condition and its associated complications remain a big challenge and should be highly individualized.

Human ; Male ; Adult ; Arteriovenous Malformations ; Embolization, Therapeutic ; Telangiectasia, Hereditary Hemorrhagic

No abstract available.
Heart ; Korea ; Thoracic Surgery

No abstract available.
Hong Kong* ; Rehabilitation* ; Stroke*