Intermittent cyanosis due to prominent eustachian valve in a newborn infant.

A full-term neonate presented with cyanosis only when sleeping, which was considered due to a prominent eustachian valve, directing blood flow from the inferior vena cava to the left atrium through the foramen ovale resulting in interatrial right-to-left shunting. In addition to the anatomical features, hemodynamic features shortly after birth, such as patent foramen ovale and high pulmonary vascular resistance, were also probably involved in the mechanism responsible for the patient's cyanosis. This case may provide insight into differential diagnosis for cyanotic infants.

Ulinastatin therapy in kawasaki disease.

BACKGROUND AND OBJECTIVE: Ulinastatin therapy may be an additional therapeutic approach to Kawasaki disease (KD). This study set out to determine whether primary intravenous ulinastatin therapy has more beneficial effects than intravenous immunoglobulin (IVIG) therapy in the acute phase of KD, and whether addition of ulinastatin to IVIG might improve outcomes in KD. METHODS: Patients were included in the study if they had a diagnosis of KD with a Harada's score that predicted coronary artery lesions. Subjects were selected to receive either primary ulinastatin therapy (30 000 U/kg/day for 3 days) or IVIG therapy (1 g/kg/dose) using sealed envelopes. Of the 27 study subjects, 18 were assigned to the ulinastatin group, and nine to the IVIG group. IVIG therapy could be added to ulinastatin therapy if patients experienced adverse effects of ulinastatin, were found to have complicated coronary artery lesions, or developed prolonged fever or elevated white blood cell counts or C-reactive protein levels. RESULTS: More patients receiving IVIG as primary therapy had reduced fever and C-reactive protein levels than patients receiving ulinastatin as primary therapy. Five patients in the ulinastatin group (28%) improved without additional IVIG therapy. These patients had lower white blood cell counts and C-reactive protein levels on admission. CONCLUSION: Primary ulinastatin therapy prevented coronary artery lesions in only 28% of cases of KD with a Harada's score predictive of such lesions. Primary ulinastatin therapy may not be the treatment of first choice for preventing coronary artery lesions in patients with KD.

Left ventricular diastolic performance in neonates.

BACKGROUND: The left ventricular (LV) diastolic performance of infants who were in a stable post-treatment condition in the neonatal intensive care unit was evaluated using echocardiography. METHODS AND RESULTS: The study group comprised 55 infants (Stable infant group, SI) and the parameters of LV performance were: LV propagation velocity (Vp) by color M-mode Doppler echocardiography (CMD), peak E wave, peak A wave, and the E/A ratio of transmitral flow. In a second set of measurements, a subset of 10 infants (patent ductus arteriosus (PDA) infant group, PI) were evaluated for LV diastolic performance during closure of PDA. The mean Vp in the SI was 27.2+/-7.3 cm/s and a positive correlation was observed between Vp and gestational age (r = 0.477, p = 0.0002). In the PI, Vp did not change significantly during closure of the PDA (from 23.3+/-8.2 cm/s to 27.5+/-8.4 cm/s); however, the E/Vp ratio decreased significantly with closure (from 3.14+/-0.83 to 2.12+/-0.68, p = 0.0051). CONCLUSION: The measurement of Vp by CMD can be considered a parameter for the evaluation of LV diastolic performance, even in the neonatal period. The LV diastolic performance of the infant is maintained from immediately after birth to spontaneous closure of the PDA.

When should pulmonary artery angioplasty be performed for Fontan candidates with pulmonary coarctation? Two cases of pulmonary artery angioplasty with the Blalock-Taussig shunt on pump in neonates.

We performed concomitant pulmonary artery angioplasty and the Blalock-Taussig shunt under median sternotomy and cardiopulmonary bypass at the neonatal stage in Fontan candidates with pulmonary coarctation to obtain optimum pulmonary circulation. This surgical strategy realized appropriate early growth of the pulmonary artery necessary for the Fontan operation.

Co-occurrence of coarctation of the aorta and hypospadias in smaller twins in monochorionic pregnancies: two case reports.

We reconstructed of the aortic arches of two unrelated boys, who were the smaller twin in monochorionic pregnancies, because they had coarctation of the aorta (CoA). They both had hypospadias. The co-twins, the larger twins, had neither CoA nor hypospadias. Although CoA and hypospadias both have been associated separately with monozygotic pregnancy, this is the first report of CoA and hypospadias occurring together. These cases indicate that epigenetic factors such as unbalanced fetal circulation in monochorionic pregnancy may be involved in co-occurrence of CoA and hypospadias.

Who is at risk for cardiac events in young patients with long QT syndrome?

The objective of this study was to determine who is at risk for cardiac events among young patients with long QT syndrome (LQTS) with or without a past history of LQTS-related cardiac events. The subjects were young patients with LQTS who had visited one of 36 hospitals from January 1997 to August 2000 in Japan. To predict the risk factors for cardiac events, stepwise regression analyses were performed for a total of 197 cases. There were 7 of 129 cases (5%) without a past history and 32 of the 68 (47%) cases with a past history of LQTS-related cardiac events that experienced new events after diagnosis (p<0.0001). Patients with a family history showed a higher incidence of symptoms both before and after diagnosis than patients with sporadic occurrence. Analyses revealed that noncompliance with medication and a lower age at diagnosis were significant predictors for the group with a past history. A negative predictive value <4 points was 100% in the group without a past history. To prevent future cardiac events, compliance with medication must be improved in those with a past history. A total LQTS score <4 points was useful to predict the absence of cardiac events in the group without a past history.

Peripheral blood eosinophilia and eosinophil accumulation in coronary microvessels in acute Kawasaki disease.

BACKGROUND: Early stage Kawasaki disease (KD) histopathology includes perivasculitis and vasculitis of the microvessels. The lesions then extend to larger vessels. Therefore the analysis of microvessel lesions is important to better understand the initial pathogenesis of KD vasculitis. METHODS AND RESULTS: We studied epicardial microvessel lesions (<50 microm) and aneurysm lesions of paraffin-embedded cardiac tissues from 4 Japanese patients who died 7 to 22 days after KD onset. The cellular composition in the microvessel lesions was different from that in coronary aneurysm lesions; eosinophils were preferentially accumulated in the microvessel lesions. The average population of eosinophils was 16% of total infiltrated cells in the microvessel lesions, whereas it was 3% in the intima of aneurysm walls. We examined peripheral blood eosinophil cell counts in 95 KD patients and 95 febrile age-matched controls. Baseline eosinophil cell counts in KD patients were higher than those in febrile control patients (361 +/- 441 65 +/- 133; < 0.0001). Eosinophilia (>350 cells/microl) before therapy was documented in 36% of KD patients, but in only 4% of febrile controls ( < 0.0001). Sixty-six KD patients (69%) developed eosinophilia within 2 weeks of illness. CONCLUSIONS: Because the numbers of circulating eosinophils in the body are tightly regulated, eosinophil accumulation in blood or tissues may reflect the host's immune response against KD related antigen(s).