Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia.
K W West, K Bengston, F J Rescorla, W A Engle, and J L Grosfeld
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Abstract
One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.
ECMO, at least in Canada, is now rarely used for patients with CDH. This is likely due to the excellent advancements in neonatal care and ventilatory strategies such as HFO. ECMO is much more liberally used in the United States. Institutions with high volumes of CDH and ECMO use generally show pretty good outcomes, but it is unclear how many of these infants would have also done well without the use of ECMO. In our institution ECMO for CDH is really a "last option" if all else fails. I participated in 2 repairs on ECMO during fellowship. One "early" at about 72 hours and one "late" at 7 days into the run. We ran a controlled anticoagulation protocol and did not have issues with hemorrhage during the repairs. Neither infant survived to decannulation.
ECMO support in CHD is essential to provide post operative care in patient after CHD surgery or after cardiac arrest if considered as ECPR. Success of surgery on ECMO depend obviously to the indication of ECMO, it the ECMO is used as preoperative circulatory/respiratory support then if the condition is due to unrepaired CHD the surgical procedure usually allowed good recovery and weaning. If ECMO is deployed after surgery for CHD and if an anatomical residual lesion that can explain the need of support are found; the surgery to aiming to resolve the residual lesion is mandatory because it will be unlikely that you will able to wean the support.
You can find extensive information about outcomes for the extracorporeal life support Organization at http://www.elso.org/Home.aspx.
It is a point of reference for all in the field of ECMO and life support
By the way if you look on ECMO support in general this is a very good article for Hospital for Sick Children in Toronto.
Evolution of technology, establishment of program, and clinical outcomes in pediatric extracorporeal membrane oxygenation: the "sickkids" experience.
Kotani Y, Honjo O, Davey L, Chetan D, Guerguerian AM, Gruenwald C.
Technological development has had a tremendous impact on the management of patients who require extracorporeal membrane oxygenation (ECMO). Team development and education are a vital component of a successful extracorporeal life support (ECLS) Program to reduce complications and subsequently improve clinical outcomes. We sought to review the evolution in technology, importance of team development and training, and report our experience at The Hospital for Sick Children, Toronto. There were a total of 576 ECMO runs in 534 patients (42 repeat ECMO runs) between January 1988 and June 2012. The use of ECMO for cardiac disease has increased in the last decade due to an expanded indication for ECMO in patients with single-ventricle physiology. Cardiac ECMO still remains a challenge in terms of survival (177/392, 45%). Although development of an ECLS program and team education facilitated extracorporeal cardiopulmonary resuscitation, clinical outcomes were not satisfactory (survival, 33%). The most common complications were hemorrhagic (13.8%), followed by renal (10.6%) and pulmonary dysfunction (6.9%). Advances in technology made management during ECMO safer, and the mechanical complications related to the ECMO system were 6.1%, including circuit changes due to thrombus formation, cannula repositioning, or optimization of size.