No abstract available.
Fontan Procedure*

No abstract available.
Fontan Procedure*

No abstract available.
Fontan Procedure*

No abstract available.
Fontan Procedure*

No abstract available.
Fontan Procedure* ; Heart*

No abstract available.
Fontan Procedure* ; Heart Ventricles*

BACKGROUND: Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan. METHODS: In a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication. RESULTS: In patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 +/- 0.14 and 0.59 +/- 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 +/- 0.26, LPA PI = 0.98 +/- 0.27) and in normal individuals (RPA PI = 1.59 +/- 0.12, LPA PI = 1.64 +/- 0.17) for both branches (p = 0.000). CONCLUSION: Using a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.
Echocardiography* ; Fontan Procedure ; Heart ; Humans ; Pulmonary Artery*

As the operative mortality has diminished and the number of survivors has increased after Fontan procedure, morbidities related to the unnatural physiology of cavopulmonary flow have developed. One of the complications by the hemodynamic derangement after Fontan procedure is a protein-losing enteropathy. This is a rare but life-threatening complication after the Fontan operation. Treatment strategies are highly variable. But, reports on successful management are limited. We experienced three cases of protein-losing enteropathy after the Fontan operation. We report that the conversion to the total extracardiac or intracardiac cavopulmonary connection with fenestration is a satisfactory treatment modality for protein- losing enteropathy after the Fontan operation.
Fontan Procedure* ; Hemodynamics ; Humans ; Mortality ; Physiology ; Protein-Losing Enteropathies* ; Survivors

The definition of complex transposition of the great arteries is not certain. Usually, the heart of simple transposition of the great arteries with atrioventricular valve anomaly, subpulmonary stenosis (left ventricular outflow tract obstruction; LVOTO), and coarctation (COA) seems to be the complex transposition of the great arteries. In this paper, the hearts with severe AV valve anomaly, hypoplastic ventricle as the candidate of Fontan procedure were excluded. Several authors published the result of complex TGA surgery worse than that of simple TGA, but recently, many authors emphasized that the result of complex TGA surgery was not worse even in the case including VSD, PS, and COA. This improvement can be achieved by precise anatomic diagnosis, and well designed surgical plan and better postoperative care.
Arteries* ; Constriction, Pathologic ; Diagnosis ; Fontan Procedure ; Heart ; Postoperative Care

Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients diagnosed with plastic bronchitis accompanied with a different underlying disease, which were treated with inhaled corticosteroid and low-dose oral clarithromycin.
Asthma ; Bronchitis ; Bronchoscopes ; Child ; Clarithromycin ; Fontan Procedure ; Humans ; Plastics