A late diagnosis of Pseudohypoaldosteronism type I in an infant with hypoplastic left heart syndrome presenting with failure to thrive

Abstract Pseudohypoaldosteronism type I is caused by a peripheral resistance to aldosterone and can present with electrolyte abnormalities, poor growth, or dehydration. Although a rare disease, several case reports have been published regarding Pseudohypoaldosteronism type I in neonates and infants. We report a case of failure to thrive and hyponatremia in an infant with hypoplastic left heart syndrome who was subsequently found to have Pseudohypoaldosteronism type I.

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Cerebral arterial gas embolism in a patient with hypoplastic left heart syndrome treated with emergent hyperbaric oxygen: case report

Undersea and Hyperbaric Medicine ◽

10.22462/03.20.2020.13 ◽

2020 ◽

pp. 431-434

Author(s):

Mark Binkly ◽

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Matthew Kelly ◽

Kevin Hardy ◽

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...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Hyperbaric Oxygen ◽

Single Ventricle ◽

Case Reports ◽

Pulmonary Arteries ◽

Vena Cava ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Small Areas ◽

Left Heart Syndrome

A 30-year-old female with a history of seizure disorder and hypoplastic left heart syndrome treated with a Norwood procedure in 1986 followed by a modified non-fenestrated Fontan (Left SVC to IVC to pulmonary arteries) with a known baffle leak presented to the emergency department. On day of presentation, the patient became unresponsive, with perioral cyanosis, rightward gaze and a left facial droop near the end of a platelet transfusion. An emergent non-contrast head CT revealed intracranial air in the right MCA distribution. She was taken to the hyperbaric chamber and was treated with a U.S. Navy Table 6 in a multiplace chamber with no extensions. Ten minutes into the treatment patient became more alert and spontaneously asked questions. The following day she was treated with a U.S. Navy Table 5. Patient had repeat CT of the head, which showed resolution of intracerebral gas and small areas of ischemia in right frontal lobe and right caudate. On hospital day five neurologic exam was normal, with 5/5 strength and no residual deficits. Treating the patient was a concern because patient has a single ventricle, in which the pulmonary artery is connected directly to the vena cava. There is very little data regarding the effects of hyperbaric oxygen (HBO2) therapy on single-ventricle physiology. Only two case reports of three pediatric patients treated with HBO2 for CAGE in a similar setting are known. In these cases the patients had improvements in their symptoms following HBO2. These cases and ours indicate HBO2 is feasible and indicated for CAGE in patients with cyanotic congenital heart disease.

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Feeding, growth, nutrition, and optimal interstage surveillance for infants with hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951111001600 ◽

2011 ◽

Vol 21 (S2) ◽

pp. 59-64 ◽

Cited By ~ 36

Author(s):

David A. Hehir ◽

David S. Cooper ◽

Elizabeth M. Walters ◽

Nancy S. Ghanayem

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Home Monitoring ◽

Failure To Thrive ◽

Normal Growth ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Interstage Mortality ◽

Annual International Symposium ◽

Stage 1 ◽

Left Heart Syndrome

AbstractImprovement in operative survival of patients with hypoplastic left heart syndrome has led to increasing emphasis on prevention of interstage mortality. Many centres have improved interstage results through programmes of home monitoring following discharge after the Norwood (Stage 1) operation. Experience with heightened interstage surveillance has identified failure to thrive during infancy as a modifiable risk factor for this population, one that has been linked to concerning outcomes at subsequent palliative surgeries. Ensuring normal growth as an infant has thus become a priority of management of patients with functionally univentricular hearts. Herein, we review the existing evidence for best practices in interstage surveillance and optimal nutrition in infants with functionally univentricular hearts. In addition, we highlight data presented at HeartWeek 2011, from Cardiology 2011, the 15th Annual Update on Pediatric and Congenital Cardiovascular Disease, and the 11th Annual International Symposium on Congenital Heart Disease.

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