Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management.

OBJECTIVE: To determine the attitudes of pediatric residents and nurses towards fetal/neonatal management of hypoplastic left heart syndrome (HLHS), and their basis. METHODS: Pediatric residents and nurses from three cardiac centers completed a survey with hypothetical scenarios in which their own fetuses or newborns had HLHS. While Institution A performs many HLHS surgeries, Institution C performs very few. RESULTS: A total of 43% of residents and 50% of nurses would terminate an affected pregnancy. More experience (4 to 7 years, p = 0.04; >7 years, p = 0.05) and employment at institution C (p = 0.04) predicted termination. Expected better quality of life (QOL) (p = 0.02) and five-year survival >50% (p = 0.06) predicted not terminating. Postnatally, 48% of residents and 68% of nurses would choose, or seriously consider, comfort care. Marriage (p = 0.04) and more experience (4 to 7 years, p = 0.04; >7 years, p = 0.02) predicted choosing comfort care. Asian/Pacific Islander descent (p = 0.01) and expected 5-year survival >50% (p = 0.02) predicted choosing surgery. CONCLUSIONS: Approximately one-half of the pediatric residents and nurses surveyed would choose termination of pregnancy or seriously consider declining neonatal surgery, if their own fetus or infant had HLHS. These attitudes reflect perceptions of long-term QOL and survival. These attitudes may be of interest to caregivers who care for HLHS patients.

Critical tricuspid insufficiency due to papillary muscle rupture. A result of prenatal hypoxic insult.

Fatal tricuspid insufficiency secondary to papillary muscle rupture due to prenatal hypoxic insult occurred in a full-term newborn. The diagnosis of flail tricuspid valve should be considered when fetal distress is encountered in a newborn with persistent hypoxemia. Prenatal diagnosis of this condition combined with prompt delivery, prostaglandin E1 therapy, and possible surgical repair of the tricuspid valve may improve chances of survival.

Spontaneous regression of cardiac rhabdomyoma in tuberous sclerosis.

Life-threatening cardiac rhabdomyoma in a newborn infant regressed spontaneously within a 5 month period. Since cardiac surgery for rhabdomyoma is dangerous in infancy, medical management should be considered if symptomatology is not severe. When a fetal arrhythmia is diagnosed, antenatal ultrasound examination for presence of cardiac tumors is warranted.

Effect of endogenous opioid blockade on fetal cardiovascular and sympathoadrenal responses to hypoxemia induced by umbilical cord constriction.

The cardiovascular and plasma catecholamine responses to hypoxemia following endogenous opioid blockade were evaluated in 18 late gestation fetal lambs (0.75-0.83 gestation) in utero. Hypoxemia was produced by progressive constriction of the umbilical cord with an inflatable silicone rubber cuff while fetal heart rate, blood pressure, and arterial blood gases were monitored. Fetal arterial blood samples were obtained for plasma catecholamine analysis using a radioenzymatic method. The animals were divided into 4 experimental groups. Control animals during normoxemia and following hypoxemia are represented by groups I and III, respectively. During umbilical constriction, arterial oxygen tension declined from 21.4 +/- 0.3 to 13.4 +/- 0.3 torr (p less than 0.001, mean +/- SE) and was accompanied by an increase in blood pressure (44 +/- 1 to 53 +/- 1 torr), decrease in heart rate (176 +/- 4 to 114 +/- 4 beats/min) and rise in plasma norepinephrine (437 +/- 90 to 3,410 +/- 617 pg/ml) and epinephrine (53 +/- 10 to 1,074 +/- 325 pg/ml). Naloxone infusion had no significant effect on the fetus during either normoxemic (group II) or hypoxemic conditions (group IV). Plasma catecholamines rose less in group IV than in hypoxemic control fetuses (norepinephrine: 2,407 +/- 406 vs. 3,410 +/- 617 pg/ml; epinephrine: 966 +/- 641 vs. 1,074 +/- 305 pg/ml), but the differences were not significant (p greater than 0.2). It is concluded that endogenous opioids do not play a major role in the modulation of cardiovascular and sympathoadrenal adaptations to moderate hypoxemia in the fetus.

Fetal cardiovascular and breathing movement responses to endogenous opiates.

Endogenous opiates have been shown to alter cardiopulmonary activity in a variety of animal models. The present investigation in fetal sheep evaluate the response of heart rate, blood pressure and breathing movements (FBM) to central nervous and intravenous meth-enkephalin and beta-endorphin. Marked bradycardia was noted within 3-5 s of intravenous meth-enkephalin administration and lasted less than 10-15 s. An 84.8 +/- 19.0% prolongation in cardiac cycle length was observed after 20 nmol/kg meth-enkephalin (p less than 0.02). In contrast, there was no significant change in heart rate following 2-50 nmol/kg beta-endorphin. The meth-enkephalin-induced slowing of heart rate was virtually eliminated by autonomic blockade with atropine or hexamethonium but merely partially blunted by naloxone. FBM were not altered by intravenous administration of either opiate. However, both meth-enkephalin and beta-endorphin produced a dramatic increase in FBM following injection into the cisterna magna. Prior to opioid administration, FBM were noted 11 +/- 1.4% of the time. Intracisternal opiates resulted in a 43.9 +/- 10.1% incidence of FBM for up to 60 min. Parasympathetic blockade with atropine had no effect on the pattern of FBM following intracisternal opiate administration whereas naloxone terminated the increased respiratory activity within 1 min. These data suggest a potential role for endogenous opiates in the modulation of fetal cardiorespiratory function.