Hypoplastic left heart syndrome: from fetus to fontan

Abstract

The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4-6 months of life, and finally total cavopulmonary connection (Fontan) at 2-4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.

Outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants

“…That is why transcatheter BAS is usually ineffective in these cases. [1,5,14] We performed BAS in five patients with some success; however, the recurrent restriction rate was high. During the inter-stage period, we observed progressive residual or recurrent atrial septal restriction causing severe pulmonary venous hypertension, heart failure, and failure to thrive.…”

“…Although compassionate care or heart transplantation are options for some centers and countries, eventual Fontan tract is the current management strategy in many centers. [1,2] Despite considerable improvements have been achieved in Norwood Stage I procedure owing to the improvements in intensive care unit facilities and some surgical modifications such as Sano shunt, beating heart techniques, and Dunk technique in the last two decades, early mortality still remains as high as 20 to 40% with significant morbidity. [1][2][3][4] The hybrid procedure (placement of bilateral pulmonary artery [PA] bands and a ductal stent with or without atrial septal manipulation) has emerged in the last decades as an alternative to Stage I palliation for neonates with HLHS.…”

“…[1,2] Despite considerable improvements have been achieved in Norwood Stage I procedure owing to the improvements in intensive care unit facilities and some surgical modifications such as Sano shunt, beating heart techniques, and Dunk technique in the last two decades, early mortality still remains as high as 20 to 40% with significant morbidity. [1][2][3][4] The hybrid procedure (placement of bilateral pulmonary artery [PA] bands and a ductal stent with or without atrial septal manipulation) has emerged in the last decades as an alternative to Stage I palliation for neonates with HLHS. [5] The early success with the hybrid approach reported by Akintürk et al [6] and Galantowicz et al [7] have prompted the increasing use of this strategy to minimize the deleterious impact of the conventional surgical intervention in high-risk patients.…”

Left heart hypoplasia operated using double pulmonary arterial banding with double arterial duct stenting: A case report

“…The exact cause of the disease is unknown, but it has been postulated that it has a multifactorial transmission. There are family cases transmitted recessively and autosomally; in 5-15% of cases, HLHS is included in genetic syndromes, such as Turner, Noonan, Smith-Lemli-Opitz or Holt-Oram, Edwards (trisomy 18), Patau (trisomy 13), Jacobson (chromosome deletion 11q), Rubinstein-Taybi and partial trisomy 9 (1,8,9).…”

Sinus Superflex DS© early collapse in systemic arterial duct

“…Hybrid approach for postnatal treatment of hypoplastic left heart syndrome has been reported more than 10 years ago [1][2][3] . Instead of continuous infusion of prostaglandin, duct stenting is usually performed for remaining the arterial duct widely open.…”