Critical congenital heart disease in neonates: a review article

: Critical congenital heart defects (CCHDs) are serious malformations which remain to be an important cause of neonatal mortality and morbidity. The clinical presentations of CCHD are shock, cyanosis or respiratory distress which may be similar to that of other neonatal conditions. Failure to diagnose these conditions early on after birth may result acute cardiovascular collapse and death. Screening with routine pulse oximetry is efficient in distinguishing newborns with CCHD and other hypoxemic illnesses, which may be otherwise be potentially life-threatening. If the cardiovascular system cannot be observed by echocardiography, then treatment with continuous prostaglandin-E1 (PGE1) infusion should be started in any newborn whose condition deteriorates in the first few days of life. This review aims to provide a concise summary on the presentation and management of various CCHDs and to emphasize on the role of timely diagnosis in the management.

COST-EFFECTIVENESS OF PULSE OXIMETRY SCREENING FOR CRITICAL CONGENITAL HEART DEFECTS IN ONTARIO

BACKGROUND Critical congenital heart defects (CCHDs) are a leading cause of morbidity and mortality in newborns, and late diagnosis is associated with mortality and worse outcomes. Many jurisdictions in the USA and elsewhere have implemented routine pulse oximetry screening (POS) for CCHD, which the Canadian Paediatric Society has recently endorsed. Cost-effective analyses in USA and Europe support this approach, but the geographical setting of Ontario in relation to its vast yet sparsely populated regions presents unique challenges with regard to POS implementation. OBJECTIVES To estimate the cost-effectiveness of POS for CCHD in the context of its implementation in Ontario, Canada. DESIGN/METHODS A cost-effectiveness analysis using a Markov model was conducted inputting values derived from an extensive review of literature, and using relevant local databases. The base-case was a 24-hour clinically stable infant born in Ontario. The model employed the healthcare payer (ministry of health) perspective and a life-time horizon. A number of mutually exclusive health states were created, representative of the natural course of CCHDs. The strategies compared were routine pulse oximetry screening versus no screening. Outcome measures, all discounted 1.5%, were quality-adjusted life months (QALMs), lifetime costs, and incremental cost-effectiveness ratios. An a priori threshold of CAD$4,166.67 per QALM (equivalent to CAD$50,000 per quality adjusted life year) was used. Probabilistic sensitivity analysis was conducted using multiple simulations of the model within expected range of variables included in the model. RESULTS The incremental cost of performing POS was estimated to be $27.27 per individual, with a gain of 0.02455 QALMs (Table 1). This yielded an incremental cost-effectiveness ratio (ICER), [Δ Cost / Δ QALMs] of CAD$1,110.79, well below the pre-determined threshold for cost-effectiveness. A probabilistic sensitivity analysis estimated a 93% chance of routine implementation of POS of being cost-effective, with majority of simulated ICERs lying below the threshold of acceptability (Figure 1). CONCLUSION Routine implementation of POS for CCHD is expected to be cost-effective with a high degree of certainty. Further validation of this model may be conducted following implementation to confirm these findings based on local population data.

Pulse Oximetry During the First 24 Hours as a Screening Tool For Congenital Heart Defects

Introduction: Although screening for congenital heart defects (CHD) relies mainly on antenatal ultrasonography and clinical examination after birth, life-threatening cardiac malformations are often not diagnosed before the patient is discharged. Aim: To assess the use of routine pulse oximetry in the delivery room and at 24 hours postpartum, and to study its feasibility as a screening test for CHD. Material and Methods: In this prospective study, all infants born in “Cuza Voda” Maternity Hospital, Iasi, Romania, were enrolled over a thirteen-month period. Preductal oximetry was assessed during the first hour, and postductal oximetry was evaluated at twenty-four hours postpartum. Data were then analyzed to establish the sensitivity and specificity of pulse oximetry, as a screening test for CHD. Results: 5406 infants were included in the study, with a mean gestational age of 38.2 weeks and a mean birth weight of 3175 grams. During the first minute, blood oxygen saturation varied between 40% and 90% and at 24 hours of life, it ranged between 90% and 100%. Following oximetry assessment, 14 infants with critical CHD were identified. Blood oxygen saturation values in infants with CHD were lower throughout the entire period of evaluation. Pulse oximetry had good sensitivity and specificity at 1 hour (Se=87.5%, Sp=95.5%) and 24 hours (Se=92.5%, Sp=97.4%) for the diagnosis of CHD. Blood oxygen saturation values at one minute, 1 hour and 24 hours are strong discriminative parameters for the early diagnosis of CHD. Conclusion: Routine pulse oximetry during the first 24 hours postpartum represents an early indicator of CHD to facilitate timely intervention. Pulse oximetry provides excellent sensitivity and specificity and has tremendous potential as a standard screening test for CHD during the first 24 hours of life.