TI: A new protocol for the perinatal management of patients with congenital diaphragmatic hernia with severe hypoplastic lungs and its clinical application.

AU: Senzaki-H; Tamura-M; Sakakihara-Y; Hishi-T

SO: Acta-Paediatr-Jpn. 1994 Oct; 36(5): 497-500

PY: 1994

AB: It is prerequisite for the pre-operative management of patients with congenital diaphragmatic hernia (CDH) to avoid the factors that increase pulmonary vascular resistance, because such patients easily fall into the state of persistent fetal circulation (PFC). In this paper, a new protocol is proposed for the perinatal management of CDH patients to prevent the PFC which is facilitated by the enlargement of hernia and the deviation of mediastinum caused by spontaneous breathing just after birth. We also describe the usefulness of this protocol based on our experience in which we successfully treated a patient with CDH with severe hypoplastic lungs that were diagnosed antenatally by ultrasonographic examination. In treating the CDH patient with severe hypoplastic lungs, it is effective for the respiratory control and the prevention of PFC to administer morphine and pancuronium to the neonate through the umbilical vein before the resection of the umbilical cord. In the case of Cesarean section, in addition to the direct administration of morphine to the patient, administration of morphine to the patient's mother just before the delivery is more effective to prevent PFC, which can be easily induced by the initial resuscitation at birth.

TI: Acute heart failure and acute renal failure in Kawasaki disease.

AU: Senzaki-H; Suda-M; Noma-S; Kawaguchi-H; Sakakihara-Y; Hishi-T

SO: Acta-Paediatr-Jpn. 1994 Aug; 36(4): 443-7

PY: 1994

AB: Acute renal failure and acute heart failure are rare in Kawasaki disease. We experienced two patients with Kawasaki disease who presented acute renal failure and acute heart failure. These two patients gave us an important insight into the understanding of water balance and fluid therapy in Kawasaki disease. One patients showed acute prerenal failure due to fluid exudation from the intravascular to the extravascular space, and subsequent acute heart failure. The other patient showed acute heart failure caused by fluid infusion for the treatment of dehydration. It is suggested that acute renal failure could be caused by a fluid shift from the intravascular to the extravascular space in Kawasaki disease. It is also demonstrated that the reserve of cardiac function could be decreased in patients with Kawasaki disease due to myocarditis even with normal echocardiography and chest X-rays.

TI: Ventricle works as a converting organ of atrial blood flow: physiological significance of mean ventricular pressure.

AU: Senzaki-H; Hishi-T; Ueda-J; Sakakihara-Y; Kamoshita-S

SO: Acta-Paediatr-Jpn. 1994 Jun; 36(3): 239-43

PY: 1994

AB: The roles and characteristics of the ventricle were examined using mean ventricular pressure (MVP) in ventricular-vascular association. One hundred and two patients with congenital heart diseases who had undergone cardiac catheterization were studied. They were divided into five groups: Group 1, atrial septal defect without pulmonary hypertension (PH); Group 2, ventricular septal defect (VSD) without PH; Group 3, VSD with PH; Group 4, pulmonary valvular stenosis; and Group 5 as a control group. Then, we examined the relationships between mean pulmonary artery pressure (MPAP) and mean right ventricular pressure (MRVP), and also between mean systemic arterial pressure (MSAP) and mean left ventricular pressure (MLVP) among the five groups. Furthermore, we defined new indicators to express the ease of blood flow through each ventricle. They were referred to as a conductance of the right ventricle (CDR) and a conductance of the left ventricle (CDL), respectively. Then they were compared among the five groups. The values of MPAP/MRVP and MSAP/MLVP were kept constant to be about 1.3 and 1.7, respectively. Furthermore, CDR was different between each group according to the property of the pulmonary vascular bed, whereas CDL took almost the same value among the five groups. The ventricle works as a converter of atrial blood flow so that it can achieve efficient blood transport.

TI: [Superior vena cava-right atrial appendage direct anastomosis for repair of partial anomalous pulmonary venous connection to superior vena cava]

AU: Miyaji-K; Shimada-M; Sekiguchi-A; Nishimura-K; Ishizawa-A; Isoda-T; Senzaki-H; Hishitani-T

SO: Kyobu-Geka. 1994 Apr; 47(4): 307-10

PY: 1994

AB: Successful repair was performed for a 7-year-old male with a diagnosis of partial anomalous pulmonary venous connection (PAPVC) to superior vena cava (SVC) and superior sinus venosus atrial septal defect (ASD). The SVC was divided above the orifice of the anomalous pulmonary vein and the cephalad end of the SVC was anastomosed directly to the right atrial appendage. A patch was used to divert pulmonary venous flow from the orifice of the SVC through superior sinus venosus ASD into the left atrium. Postoperative course was uneventful with normal sinus rhythm. There was no evidence of vena caval or pulmonary venous obstruction. At 3-month after surgery, sinus node function was confirmed to be normal by electrophysiological study. This is useful alternative method for repair of PAPVC to high or middle SVC.

TI: Reconsideration of criteria for the Fontan operation. Influence of pulmonary artery size on postoperative hemodynamics of the Fontan operation.

AU: Senzaki-H; Isoda-T; Ishizawa-A; Hishi-T

SO: Circulation. 1994 Mar; 89(3): 1196-202

PY: 1994

AB: BACKGROUND: The outcome of the Fontan operation largely depends on the selection of patients because this procedure is a physiological correction. Among the several selection criteria for the Fontan operation, the importance of adequate size of the pulmonary artery remains controversial. In this series, in order to clarify whether the pulmonary artery size is indispensable or not as one of the selection criteria for the Fontan operation, we considered the physiological meaning of pulmonary artery size and investigated how it influenced postoperative hemodynamics of the Fontan operation. METHODS AND RESULTS: In congenital heart disease of decreasing pulmonary blood flow, 40 patients were examined for this analysis. Pulmonary artery indexes (cross-sectional area of the right and left pulmonary arteries divided by body surface area) were measured as the expression of pulmonary artery size, and the relations of pulmonary artery index (PAI) to pulmonary vascular resistance (Rp) and compliance (Cp) were studied. There was no significant correlation between PAI and Rp, whereas a significant correlation was found between PAI and Cp (r = .71, P = .001). Furthermore, Cp influenced postoperative hemodynamics of the Fontan operation by affecting the peak central venous pressure (pCVP) and total impedance, which was the afterload to the ventricle. Impedance increased abruptly when PAI was < approximately 100 mm2/m2. CONCLUSIONS: The smaller pulmonary artery size causes more disadvantageous hemodynamics after the Fontan operation, with resultant effects of the rise in pCVP and the increase in afterload to the single ventricle.

TI: [Relationship between deep body temperature and hemodynamics in pediatric patients with congenital heart disease]

AU: Senzaki-H; Isoda-T; Ishizawa-A; Hishi-T

SO: Nippon-Kyobu-Geka-Gakkai-Zasshi. 1994 Jan; 42(1): 56-60

PY: 1994

AB: Relationship of the abdomen-sole deep body temperature difference (DBT) to the hemodynamics was examined in patients with congenital heart disease. Hemodynamic data were obtained by routine cardiac catheter examination, and DBT was measured during the catheterization. Significant positive correlation was found between DBT and systemic-pulmonary flow ratio (Qp/Qs) (r = 0.85, p = 0.001). DBT was maintained below 2 C degrees in almost every patient when the Qp/Qs was less than 2, whereas DBT rose rapidly when the Qp/Qs was more than 2. Furthermore, systemic blood flow and arterial-venous oxygen difference ranged widely within the normal values when DBT was less than 2 C degrees. On the other hand, when DBT was more than 2 C degrees, systemic blood flow and arterial-venous oxygen difference tended to show fixed values which were around the normal upper or lower limits. This means that systemic circulation is marginally adapted when the Qp/Qs is more than 2. The measurement of deep body temperature difference is noninvasive, easy and useful as a circulatory monitor in pediatric patients with congenital heart disease.

TI: Reconsideration of criteria for the Fontan operation. Influence of pulmonary artery size on postoperative hemodynamics of the Fontan operation.

AU: Senzaki-H; Isoda-T; Ishizawa-A; Hishi-T

SO: Circulation. 1994 Jan; 89(1): 266-71

PY: 1994

AB: BACKGROUND: The outcome of the Fontan operation largely depends on patient selection because this procedure is a physiological correction. Among the several selection criteria for the Fontan operation, the importance of adequate size of the pulmonary artery remains controversial. To clarify whether or not pulmonary artery size is indispensable as one of the selection criteria for the Fontan operation, we considered the physiological importance of pulmonary artery size and investigated how pulmonary artery size influenced postoperative hemodynamics of the Fontan operation. METHODS AND RESULTS: In congenital heart disease of decreasing pulmonary blood flow, 40 patients were examined for this analysis. Pulmonary artery indexes (cross-sectional area of the right and left pulmonary arteries divided by body surface area) were measured as the expression of pulmonary artery size, and the relations of pulmonary artery index (PAI) to pulmonary vascular resistance (Rp) and compliance (Cp) were studied. There was no significant correlation between PAI and Rp, whereas a significant correlation was found between PAI and Cp (r = .71, P = .001). Furthermore, Cp influenced postoperative hemodynamics of the Fontan operation by affecting peak central venous pressure and total impedance, which was the afterload to the ventricle. Impedance increased abruptly when PAI was less than approximately 100 mm2/m2. CONCLUSIONS: The smaller pulmonary artery size is hemodynamically disadvantageous after the Fontan operation, with resultant rise in peak control venous pressure and increased afterload to the single ventricle.

TI:Circadian rhythm of the autonomic nervous system in long QT syndrome.

AU:Kato-H; Yanagawa-Y

SO:Acta-Paediatr-Jpn. 1994 Apr; 36(2): 176-82

PY:1994

AB: The circadian changes in the activity of the autonomic nervous system in a group showing long QT duration in electrocardiogram (ECG) were studied in order to differentiate symptomatic congenital long QT syndrome from asymptomatic. The asymptomatic group presented only long QT duration (QTc > 0.46). Seven girls and two boys, including two subjects experiencing syncope of non-neurological origins, were examined by using heart rate (HR) power spectrum analysis. In three subjects, the peak of the high frequency band, indicating the parasympathetic activity, disappeared during night-time (sleep), which means the possibility of a high risk of sudden cardiac death. In two of three subjects, moreover, the averaged sympathetic activity during daytime was significantly increased compared to that during night-time, in addition to the abrupt increase of sympathetic activity in the morning. These two subjects, in which one boy had a family history of Romano-Ward syndrome, were the same people experiencing the syncope attack. We succeeded in objectively elucidating that congenital long QT syndrome is adrenergic-dependent, and suggest that HR power spectrum analysis may be of value in distinguishing symptomatic congenital long QT syndrome from asymptomatic showing only long QT duration.

TI: Erythromycin improves gastrointestinal motility in extremely low birthweight infants.

Kubota-M; Nakamura-T; Motokura-T; Mori-S; Nishida-A

SO: Acta-Paediatr-Jpn. 1994 Apr; 36(2): 198-201

PY:1994

AB: Erythromycin (EM) was administered to five extremely low birthweight infants (ELBWI) with delayed enteral feeding to evaluate the clinical effect on severely impaired gastrointestinal motility. Five patients studied responded well to EM administration without any adverse effects during the course. Four patients were given 15-30 mg/kg per day EM intravenously as a loading and thereafter 3-5 mg/kg per day as a maintenance dose. One patient responded well without loading. The infants could be fed enterally 4, 5, 6, 4 and 2 days after the initiation of EM administration, respectively. Erythromycin administration is a safe and useful way to facilitate gastrointestinal motility in ELBWI who require prolonged ventilator support with an increased risk for nutrient deprivation.

Edited by Toshio Hishi