Pediatric Cardiology and Cardiac Surgery
Vol.26 No.4 2010 (338-344)

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Fumi Mashiyama,1) Nobuo Momoi,1) Kisei Endo,1) Yoshimichi Aoyagi,1) Masaki Mitomo,1) Yutaka Fukuda,1) Takashi Ono,2) Masato Endo3) and Takashi Tanaka4)

1)Department of Pediatrics, Fukushima Medical University, 2)Department of Pediatric Cardiovascular Surgery, Southern Tohoku General Hospital, Fukushima, 3)Department of Health and Social Services, Tohoku Bunka Gakuen University, and 4)Department of Cardiology, Miyagi Children’s Hospital, Miyagi, Japan


We present the case of a patient with pulmonary atresia with intact ventricular septum (PAIVS) whose treatment strategy was changed because of the disappearance of sinusoidal communications. Due to sinusoidal communications between 3 coronary vessels and a hypoplastic right ventricular cavity in the neonatal cardiac catheterization, the patient underwent a Blalock-Taussig shunt at 16 days of age as the first palliation for the staged Fontan operation. However, a cardiac catheterization in the next year revealed the disappearance of sinusoidal communications, and the patient underwent a percutaneous transluminal pulmonary valvuloplasty using a guidewire perforation for the biventricular repair. Although the right ventricular size was gradually enlarged, it was inadequate for biventricular repair. The patient underwent a one and one-half ventricular repair at 6 years of age. Evaluation one year after the operation revealed adequate coronary circulation, normal ventricular wall motion, and low right atrium pressure. The patient, now at 8 years of age, has had no arrhythmic history and is being followed up without medication. Presence or absence of large sinusoidal communications in PAIVS is a critical factor for the determination of a therapeutic strategy. In addition, it is important to note the diversity and the change with age of sinusoidal communications.