Sleep Disordered Breathing in Children With Single Ventricle Physiology

2014-07-23 21:48:47 | BioPortfolio


This is an exploratory study designed to evaluate the incidence of, and to quantify sleep disordered breathing following stage I Norwood reconstructive surgery. Sleep disordered breathing will be correlated with:

1. Elevations in pulmonary vasculature resistance at the time of Stage II surgery.

2. Risks of death


This is an exploratory study designed to evaluate the incidence of, and to quantify sleep disordered breathing following stage I Norwood reconstructive surgery. Sleep disordered breathing will be correlated with:

1. Elevations in pulmonary vasculature resistance at the time of Stage II surgery.

2. Risks of death

Children with single ventricle physiology are exquisitely sensitive to alterations in pulmonary vascular resistance. Following their first operative repair (stage I Norwood), performed in their first week of life, pulmonary and systemic circulations are in parallel rather than series. As such, elevations in pulmonary vascular resistance can result in severe arterial desaturation. Additionally, elevated pre-operative pulmonary artery pressure is directly correlated with poor survival following the third and final operative repair (stage III Norwood, or Fontan).

Periodic breathing is a normal breathing pattern in sleeping infants. At the other end of the spectrum is sleep apnea. In between lies a continuum of sleep disordered breathing. Obstructive sleep apnea has an incidence of approximately 2% in children, and is associated with pulmonary and systemic hypertension. Specific studies of the incidence and effects of sleep disordered breathing in congenital heart disease are lacking. Otherwise normal children have baseline oxygen saturation in the high 90's, thereby placing them on the flat part of the oxyhemoglobin curve. But children with cyanotic congenital heart disease live with baseline oxygen saturations in the mid 70's, so that they exist on the steep part of the oxyhemoglobin dissociation curve. We hypothesize therefore that these patients are at increased risk for the hemodynamic variations occurring during apneas/hypopneas even when they are more subtle, namely during sleep disordered breathing.

We hypothesize that children who have completed stage I Norwood will experience more significant arterial desaturations during sleep associated apneic events (due to the concurrent elevation in pulmonary arterial pressure) than their normal counterparts. Additionally we hypothesize that children who experience more frequent apneic events during sleep will have elevated pre-operative pulmonary artery pressures and therefore worse outcome following stage II Norwood. Thus, we speculate that children who have completed stage I Norwood are more prone to the risks of sleep disordered breathing.

Autonomic regulation, mediated in part by aortic arch baroreceptors, is undoubtedly disrupted by the extensive surgical reconstruction required at the aortic arch during stage I Norwood palliation. Adults and children with severe sleep disordered breathing (obstructive sleep apnea) have impaired cardiac autonomic control, and increased cardiac electrical instability, with greater occurrence of ventricular arrhythmias. Apneic events place a hypoxic, mechanical and adrenergic load on the cardiovascular system thereby directly resulting in ventricular dysrhythmias. Observed late deaths following stage I Norwood are usually postulated to be secondary to fatal arrhythmias. Thus, we hypothesize that children who experience more frequent apneic events during sleep will have an increased risk of interstage mortality.

Sleep disordered breathing is a readily treatable condition in the pediatric population. Non-invasive, continuous positive airway pressure applied via a nasal mask is effective in treating sleep disordered breathing in infants. Thus, if sleep disordered breathing is identified, effective treatment is available and may reduce the risk of inter-stage mortality and adverse hemodynamics in this medically fragile population.

Study Design

Time Perspective: Prospective


Hypoplastic Left Heart Syndrome


University of Rochester
New York
United States




University of Rochester

Results (where available)

View Results


Published on BioPortfolio: 2014-07-23T21:48:47-0400

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A condition caused by underdevelopment of the whole left half of the heart. It is characterized by hypoplasia of the left cardiac chambers (HEART ATRIUM; HEART VENTRICLE), the AORTA, the AORTIC VALVE, and the MITRAL VALVE. Severe symptoms appear in early infancy when DUCTUS ARTERIOSUS closes.

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A congenital coronary vessel anomaly in which the left main CORONARY ARTERY originates from the PULMONARY ARTERY instead of from AORTA. The congenital heart defect typically results in coronary artery FISTULA; LEFT-SIDED HEART FAILURE and MITRAL VALVE INSUFFICIENCY during the first months of life.

A syndrome of persistent PULMONARY HYPERTENSION in the newborn infant (INFANT, NEWBORN) without demonstrable HEART DISEASES. This neonatal condition can be caused by severe pulmonary vasoconstriction (reactive type), hypertrophy of pulmonary arterial muscle (hypertrophic type), or abnormally developed pulmonary arterioles (hypoplastic type). The newborn patient exhibits CYANOSIS and ACIDOSIS due to the persistence of fetal circulatory pattern of right-to-left shunting of blood through a patent ductus arteriosus (DUCTUS ARTERIOSUS, PATENT) and at times a patent foramen ovale (FORAMEN OVALE, PATENT).

A condition in which the LEFT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE; MYOCARDIAL INFARCTION; and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the left ventricular wall.

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