Somatic growth failure after the Fontan operation

Abstract

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.

Comparison of somatic development and status of conduit after extracardiac Fontan operation in young and older children?

“…During the era before the Fontan operation many investigators showed that somatic development of children with chronic cyanosis was frequently inadequate compared to that of the healthy population [14][15][16]. Our results support this, with the dramatic deceleration of weight and length gain according to the percentiles after birth and before Fontan operation in all children, which is probably related to congestive heart failure and cyanosis [14][15][16].…”

“…The anastomosis between the inferior vena cava (IVC) and the pulmonary artery was established using a Gore-Tex w (W.L. Gore and Associates, Inc, Flagstaff, AZ, USA) conduit with a median diameter of 18 (16-24) mm in young children and 20 (16)(17)(18)(19)(20)(21)(22)(23)(24) mm in older children. In 40 children the operation was performed on the beating heart with normothermic cardiopulmonary bypass (median duration 91 (30-274) min).…”

“…During the era before the Fontan operation many investigators showed that somatic development of children with chronic cyanosis was frequently inadequate compared to that of the healthy population [14][15][16]. Our results support this, with the dramatic deceleration of weight and length gain according to the percentiles after birth and before Fontan operation in all children, which is probably related to congestive heart failure and cyanosis [14][15][16]. Stenborg et al described significantly improved physical growth following the total cavopulmonary connection but Cohen et al showed significantly decreased weight and length in children who had undergone Fontan operation, compared to their healthy peers [16].…”

Zespół utraty białek u pacjentów z pojedynczą komorą serca po operacji Fontana

“…U pacjentów z PLE dochodzi do zahamowania rozwoju fizycznego i utraty masy ciała [27]. Utrata białek i limfocytów oraz uogólniona reakcja zapalna towarzysząca temu zespołowi prowadzą do niedożywienia, wyniszczenia, zaburzeń wodnoelektrolitowch i upośledzenia odporności.…”

“…Takie trójglicerydy wchłaniane są bezpośrednio, przez naczynia krwionośne układu wrotnego, z pominięciem naczyń limfatycznych. Ze względu na częste wyniszczenie tych pacjentów, bardzo istotne znaczenie ma dostarczenie odpowiedniej liczby kalorii [10,27].…”