Tatsuo Katori

Moderate-Term Effect of Epoprostenol on Severe Portopulmonary Hypertension

We examined the effect of continuous intravenous infusion of epoprostenol (35 ng/kg/min) on severe portopulmonary hypertension caused by biliary atresia. Pulmonary hemodynamics improved and brain natriuretic peptide and human atrial natriureic peptide decreased to normal values during epoprostenol therapy. However, the improvement in pulmonary hemodynamics was not sufficient to permit liver transplantation. Our patient was obliged to stop epoprostenol therapy because of financial problems and epoprostenol was tapered off safely over 6 weeks.

Prenatal diagnosis of coronary artery fistula: A case report and review of literature

to diagnosis was about 1 month from birth. Although our patient had a normal Apgar score, his respiratory condition rapidly worsened over the next 3 days. The patient’s respiratory disorder was so severe that he was kept in hospital for 5 weeks; however, endoscopic laryngeal surgery was performed on the day of diagnosis, and this immediate treatment may have resulted in the good neurological prognosis at 12 months. Eighteen infantile cases of epiglottic cyst or VC have been reported in the English literature from 1997 to 2006. No malignant change has so far been reported. Regarding the treatment of VC in infants, prognosis is good if cysts are removed completely. Several studies have reported no recurrence of VC; however, insufficient resection might result in recurrence. VC should be considered in the differential diagnosis of an infant presenting with respiratory distress, particularly with stridor. In the case of persistent stridor and feeding difficulty, VC should be suspected, and flexible nasopharyngoscopy should be performed. Surgery is the treatment of choice.

Markedly Dilated Right Heart 17 Years After Initial Treatment Repaired by Total Right Ventricular Exclusion and Total Cavopulmonary Connection

The total right ventricular (RV) exclusion procedure for isolated congestive RV failure, in which most of the dilated right atrium (RA) and RV wall is resected, was first reported by Sano et al in 2002.1 This procedure improves the cardiac index2 and suppresses supraventricular/ventricular tachyarrhythmia. Using this procedure with an extracardiac conduit, we successfully treated severe heart failure characterized by a markedly dilated right heart lacking tricuspid leaflets and by recurrent tachyarrhythmia, 17 years after the patient had undergone initial definitive intracardiac repair including RV outflow tract plasty. A 22-year-old woman presented with general fatigue, dyspnea, palpitations, and loss of appetite of 6 months’ duration caused by low cardiac output. She was admitted to the University of Tokyo Hospital to undergo surgery to improve her low cardiac output. She was cyanotic at birth after an uneventful pregnancy and caesarian delivery. Cardiac catheterization performed when she was 47 days old revealed pulmonary atresia with atrial septal defect and patent ductus arteriosus. A Blalock-Taussig anastomosis was established. Subsequently, she was diagnosed with absent tricuspid valve, pulmonary atresia, and atrial septal defect. These were definitively treated surgically in 1990 when the patient was 5 years old via RV outflow tract plasty and atrial septal defect closure. Intraoperative findings included weak RV contraction and an anterior RV wall almost totally lacking myocardium. Five years after the operation, she suffered from ventricular tachycardia originating from the RV wall, which was treated by electrical cardioversion and subsequent administration of oral antiarrhythmic medication. Six months before admission, dyspnea …

Thirty-two Japanese cases of infantile hemangiomas treated with oral propranolol

Infantile hemangiomas undergo rapid growth during early infancy followed by gradual involution. Infantile hemangiomas sometimes impair vital functions or cause disfigurement. Thirty‐two Japanese patients between the ages of 1 and 4 months with proliferating infantile hemangiomas received oral propranolol on an outpatient basis. The success rate (complete or nearly complete resolution) at week 25 was 56% (18/32). Two patients dropped out because of a personal reason and moving out. Recurrence after termination of treatment was seen in six patients. Adverse events occurred in 16 patients. There were no adverse events on day 1 (initiation of treatment at a dose of 1 mg/kg per day) and day 8 (dose increase to 2 mg/kg per day). One patient was hospitalized due to pneumonia, and suspended propranolol for 26 days. Oral propranolol at 2 mg/kg per day is effective and safe in Japanese patients with infantile hemangiomas.

Oral propranolol for infantile hemangiomas beyond the proliferative phase

Infantile hemangiomas grow rapidly during infancy followed by gradual involution. After involution, residual lesions sometimes remain. Despite the prognosis for eventual involution, infantile hemangiomas often cause great psychosocial morbidity that affects patients and their parents. Oral propranolol usually induces earlier involution and redness reduction in infantile hemangiomas in the proliferative phase. However, to evaluate the effectiveness of oral propranolol for infantile hemangiomas beyond the proliferative phase is difficult because of spontaneous regression. We report five Japanese patients treated with 2 mg/kg per day of oral propranolol for infantile hemangiomas beyond the proliferative phase and compared with three untreated patients. After the oral propranolol treatment for 25 weeks, all the treated patients exhibited earlier color fading than untreated patients. Four patients reached nearly complete resolution. Adverse events occurred in three patients: cold, exanthema subitum and suspected of bronchial asthma, respectively. The propranolol treatment for the patient with suspected of bronchial asthma was suspended for 4 months. Recurrence after termination of treatment was not seen. Oral propranolol (2 mg/kg per day) is a safe and effective treatment for Japanese patients with infantile hemangiomas beyond the proliferative phase.

Prolonged growth of infantile hemangioma after pulsed dye laser and oral propranolol treatment

Infantile hemangiomas are the most common tumor of childhood and undergo rapid growth during early infancy followed by gradual involution. After involution, residual lesions sometimes remain. Oral propranolol usually induces earlier involution and redness reduction of infantile hemangiomas. However, the optimal treatment duration is unknown and infantile hemangiomas sometimes recur after cessation of treatment. We report three Japanese patients with recurrent infantile hemangiomas on their cheek. These patients were a 1‐month‐old female baby with a superficial infantile hemangioma, a 3‐month‐old female baby with a mixed infantile hemangioma and a 4‐month‐old male baby with a mixed infantile hemangioma. Two of them also received pulsed dye laser treatment. They did not reach complete or nearly complete resolution of infantile hemangiomas at week 25. These patients experienced regrowth of their infantile hemangioma after 20 months of age and took propranolol after the age of 24 months. There were no severe adverse effects. Propranolol may not only be therapeutic but also prophylactic. Patients with infantile hemangiomas who have taken oral propranolol should be followed up at least 6 months after cessation of treatment, especially infantile hemangiomas on the cheek, and those with partial response to propranolol may require close attention in prolonged growth.

Right-sided hepatic hernia of normal left lobe resembling cardiac tumor.

A 3‐year‐old boy was referred for cardiac tumor diagnosed on transthoracic echocardiography (TTE) at another hospital. The tumor appeared to be in the right atrium and obstructed inferior vena cava flow. TTE, enhanced computed tomography (CT) and angiography were done to confirm diagnosis. Subsequently, cardiac tumor was ruled out and he was diagnosed with a very rare condition of hepatic hernia containing a normal left lobe. When cardiac tumor is suspected in the right atrium on the basis of TTE, enhanced CT or magnetic resonance imaging should be done for definitive diagnosis.