Diagnosis, management, and pathophysiology of post-Fontan hypoxaemia secondary to Glenn shunt related pulmonary arteriovenous malformation.
نویسندگان
چکیده
An 8 year old child with tricuspid atresia had developed right sided pulmonary arteriovenous malformations following a previous classic Glenn procedure. These became clinically manifest immediately after Fontan conversion because of severe systemic desaturation. The pathophysiology and postoperative medical management of this case is described and related to current understanding of the aetiology of acquired pulmonary arteriovenous malformations following cavopulmonary shunt.
منابع مشابه
CASE REPORT Diagnosis, management, and pathophysiology of post-Fontan hypoxaemia secondary to Glenn shunt related pulmonary arteriovenous malformation
An 8 year old child with tricuspid atresia had developed right sided pulmonary arteriovenous malformations following a previous classic Glenn procedure. These became clinically manifest immediately after Fontan conversion because of severe systemic desaturation. The pathophysiology and postoperative medical management of this case is described and related to current understanding of the aetiolo...
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Pulmonary arteriovenous malformations (PAVM) are abnormal communications between pulmonary arteries and pulmonary veins, and most commonly congenital in nature. Although rare, it is an important consideration in cyanotic patients of unknown cause. We report 3 cases with diffuse PAVM in children with different clinical manifestations and initial diagnosis was made by transthoracic contrast echoc...
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The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arte...
متن کاملPulmonary arteriovenous fistulas in patients with left isomerism and cardiac malformations.
OVER THE PAST DECADE, VARIOUS FORMS OF cavopulmonary anastomoses (the bidirectional Glenn shunt, the hemi-Fontan procedure) have become the palliative procedure of choice for the treatment of hearts with a functionally single ventricle. Significantly, the occurrence of pulmonary arteriovenous fistulas had been noted as an important 'late' complication after construction of the classic Glenn ana...
متن کاملPulmonary arteriovenous malformations after a Fontan operation in the left isomerism and absent inferior vena cava.
OBJECTIVE To evaluate the occurrence of pulmonary arteriovenous malformations after a Fontan operation in the left isomerism and absent inferior vena cava. METHODS We retrospectively reviewed 19 patients with the left isomerism and absent inferior vena cava who underwent Fontan operations at our institution. We divided the patients into two groups: bilateral superior vena cava (group A, n=9),...
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ورودعنوان ژورنال:
- Heart
دوره 82 4 شماره
صفحات -
تاریخ انتشار 1999