Normal Ventricular and Regional Blood Flow Volumes and Native T1 Values in Healthy Japanese Children Obtained from Comprehensive Cardiovascular Magnetic Resonance Imaging.

Age-related mean and reference ranges for ventricular volumes and mass, regional blood flow measurements, and T1 values using cardiovascular magnetic resonance (CMR) imaging are yet to be established for the pediatric population. Especially in infants and toddlers, no consistent flow volume sets or T1 values have been reported. The purpose of this study was to determine the relevant normal values.Twenty-three children (aged 0.1-15.3 years) without cardiovascular diseases were included. Comprehensive CMR imaging including cine, 2-dimensional phase-contrast, and native T1 mapping, were performed. Ventricular volumes and masses, 11 sets of regional blood flow volumes, and myocardial and liver T1 values were measured. All intraclass correlation coefficient values were > 0.94, except for the right ventricular mass (0.744), myocardial (0.868) and liver T1 values (0.895), reflecting good to excellent agreement between rates.Regression analysis showed an exponential relationship between body surface area (BSA) and ventricular volumes, mass, and regional blood flow volumes (normal value = a*BSAb). Left ventricular myocardial T1 values were regressed on linear regression with age (normal value = -7.39*age + 1091), and hepatic T1 values were regressed on a quadratic function of age (normal value = 0.923*age2 -18.012*age + 613).Comparison of the 2 different methods for the same physical quantities by Bland-Altman plot showed no difference except that the right ventricular stroke volume was 1.5 mL larger than the main pulmonary trunk flow volume.This study provides the normal values for comprehensive CMR imaging in Japanese children.

Postnatal administration of systemic steroids increases severity of retinopathy in premature infants.

BACKGROUND: Postnatal systemic steroids are known to increase the risk of developing retinopathy of prematurity (ROP). However, whether the total dosage and type of postnatal systemic steroids are related to the development and severity of ROP in premature infants remains unclear. This study was conducted to identify the risk factors for ROP and investigate the relationship between photocoagulation (PC)-demanding severe ROP and the postnatal dosage of any type of systemic steroids. METHODS: A total of 75 infants born at <28 weeks of postmenstrual age (PMA) were enrolled. The number of PC procedures for ROP was evaluated as the objective variable. This study analyzed the following independent variables: gestational age; birth weight; sex; Apgar scores; duration of mechanical ventilation; duration of nasal intermittent positive pressure ventilation; mean saturation and mean oxygen concentration administration until 36 weeks of PMA; total accumulation dosage of hydrocortisone, dexamethasone, and systemic steroids; dosage number of times of erythropoietin; total dosage of red cell concentrates (RCC); incidence of necrotizing enterocolitis (NEC) and focal intestinal perforation; sepsis; bronchopulmonary dysplasia (BPD); and intraventricular hemorrhage (IVH). Logistic regression was used to estimate the relative risk (odds ratio (OR)) associated with risk factors for PC-demanding severe ROP. RESULTS: Compared with infants in the non-PC group, PC-treated infants had younger gestational age, longer mechanical ventilation periods, and higher dosage of systemic steroids and dexamethasone. Multivariate logistic regression analysis revealed total dosage of systemic steroids as the only risk factor for PC-demanding severe ROP. Based on receiver operating characteristic (ROC) curve analysis, a cutoff value of 8.95 mg/kg of postnatal systemic steroid administration was identified as a useful marker to predict PC-demanding severe ROP. CONCLUSION: By focusing on the method of systemic steroid administration and avoiding excessive doses for infants born at <28 weeks of PMA, preventing the development of severe ROP is possible.

An infant with congenital nephrogenic diabetes insipidus presenting with hypercalcemia and hyperphosphatemia.

SUMMARY: We report a male infant with congenital nephrogenic diabetes insipidus (NDI) who presented with hypercalcemia and hyperphosphatemia since birth. Serum sodium started to increase at 39 days. Although there was no polyuria, urine osmolality was 71 mOsm/kg, when serum osmolality was 296 mOsm/kg with plasma arginine vasopressin 22.5 pg/mL. He was thus diagnosed as NDI. An undetectable level of urine calcium and unsuppressed intact parathyroid hormone suggested hyperparathyroidism including calcium-sensing receptor mutations that could cause hypercalcemia-induced NDI. Polyuria became apparent after the initiation of i.v. infusion for the treatment of hypernatremia. Low calcium and low sodium formula with hypotonic fluid infusion did not correct hypernatremia, hypercalcemia, or hyperphosphatemia. Hydrochlorothiazide and subsequently added celecoxib effectively decreased urine output and corrected electrolytes abnormalities. Normal serum electrolytes were maintained after the discontinuation of low calcium formula. The genetic analysis revealed a large deletion of the arginine vasopressin receptor-2 (AVPR2) gene but no pathogenic variant in the calcium-sensing receptor (CASR) gene. Whether hypercalcemia and hyperphosphatemia were caused by dehydration alone or in combination with other mechanisms remains to be clarified. LEARNING POINTS: Congenital NDI can present with neonatal hypercalcemia and hyperphosphatemia. Hypercalcemia and hyperphosphatemia can be treated with low calcium and low sodium formula, hydration, hydrochlorothiazide, and celecoxib. Genetic testing is sometimes necessary in the differentiating diagnosis of hypercalcemia associated with NDI.

Cesarean section predominantly affects right ventricular diastolic function during the early transitional period.

BACKGROUND: Although the mode of delivery is well known to affect pulmonary function, the effects of a cesarean delivery on postnatal changes in cardiac mechanics have not been clearly defined. METHODS: To evaluate whether delivery mode influences cardiac function in the early transitional period, 42 infants delivered by cesarean section (CS) and 110 by vaginal delivery (VD) were enrolled, and they underwent serial echocardiography at 0, 1, 2, and 5 days of age. Longitudinal changes in ejection fraction (EF), fractional area change (FAC), mitral annular plane systolic excursion (MAPSE), tricuspid annular plane systolic excursion (TAPSE), Tei index, ratio of peak early diastolic flow velocity (E) to peak early diastolic annular velocity (e') (E/e'), and deceleration time (DcT) were compared between the two groups. RESULTS: FAC and DcT of both ventricles increased during the first week, whereas Tei index of each chamber decreased irrespective of delivery mode. E/e's of both ventricles were significantly higher and MAPSE was significantly lower in the CS than VD group throughout the observation period. After adjustment for the effects of birth weight, gestational age, and oxygen administration by multivariate analysis, right ventricular E/e', which reflects diastolic function of the right ventricle, was most affected by delivery mode. CONCLUSION: CS affected diastolic function of the right ventricle in the 2nd day after giving birth and did not persist. Delayed adaptation of the neonatal myocardium and/or persistence of pulmonary hypertension might explain the hemodynamic changes in neonates born by CS.

[Medium-remote Term Results of the Atrioventricular Valve Replacement with Mechanical Valve for Functional Single Ventricles].

We prospectively investigated the relation of adaptation, timing of atrioventricular valve replacement (AVVR), valve type, size, durability of replacement valve, and preoperative cardiac function with prognosis of AVVR. The subjects included 26 patients[ 15.5 years old( day 2-43 years)] with functional single ventricle who underwent AVVR at our institution between August 1996 and January 2014. Of these patients, 24 had regurgitation, whereas 2 had stenosis. Of 7 patients who died, 3 were infants who died in the postoperative acute phase, and all of them had severe heart failure at the preoperative stage. The 5-year survival rate was 67% as assessed by Kaplan-Meier curve. On univariate analysis of the preoperative data, pulmonary artery pressure (PAP), pulmonary capillary wedge pressure, age at operation, body height, and body weight were significant risk factors for death;of these, only PAP remained in the last model for multiple regression analysis. AVVR for regurgitation is supposed to reduce cardiac volume load and help improve prognosis. Atrioventricular valve plasty or replacement should be performed prior to the development of severe heart failure.

Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: effect of sildenafil, beraprost and bosentan.

The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.