Fetal cardiology: changing the definition of critical heart disease in the newborn

Abstract

Infants born with congenital heart disease (CHD) may require emergent treatment in the newborn period. These infants are likely to benefit the most from a prenatal diagnosis, which allows for optimal perinatal planning. Several cardiac centers have created guidelines for the management of these high-risk patients with CHD. This paper will review and compare several prenatal CHD classification systems with a particular focus on the most critical forms of CHD in the fetus and newborn. A contemporary definition of critical CHD is one which requires urgent intervention in the first 24 h of life to prevent death. Such cardiac interventions may be not only life saving for the infant but also decrease subsequent morbidity. Critical CHD cases may require delivery at specialized centers that can provide perinatal, obstetric, cardiology and cardiothoracic surgery care. Fetuses diagnosed in mid-gestation require detailed fetal diagnostics and serial monitoring during the prenatal period, in order to assess for ongoing changes and identify progression to a more severe cardiac status. Critical CHD may progress in utero and there is still much to be learned about how to best predict those who will require urgent neonatal interventions. Despite improved therapeutic capabilities, newborns with critical CHD continue to have significant morbidity and mortality due to compromise that begins in the delivery room. Fetal echocardiography is the best way to predict the need for specialized care at birth to improve outcome. Once the diagnosis is made of critical CHD, delivery at the proper time and in appropriate institution with specific care protocols should be initiated. More work needs to be done to better delineate the risk factors for progression of critical CHD and to determine which newborns will require specialized care. The most frequently described forms of critical CHD requiring immediate intervention include hypoplastic left heart syndrome with intact or severely restricted atrial septum, obstructed total anomalous pulmonary venous return and transposition of the great arteries with restrictive atrial septum.

Selected data from the Polish National Prenatal Cardiac Pathology Registry from the year 2016

“…Otherwise there was no differentiation such as newborn baby needs treatment immediately after childbirth or later after 2-3 weeks (Table 2). From 2012, we divided prenatal heart defects into the following groups: non-urgent, severe, critical and most severe, according to the anticipated clinical condition of the neonate and time of planned cardiac surgery [6][7][8][9] . In 2016 the most common diagnoses were severe heart defects - …”

Prenatal Diagnosis of TAPVC on Monday, Delivery of Tuesday and Cardiac Surgery at Wednesday - A Model of Perinatal Care in 3rd Trimester in Case of Fetal/Neonatal Critical Heart Defect in Tertiary Center.

“…In order to prevent the increase of pulmonary blood pressure and consequent decrease of blood saturation leading to neonatal death, neonates with TAPVC and pulmonary venous obstruction should be delivered in a tertiary center and need to undergo a surgical intervention during the first hours after birth. This implicates, that TAPVC with pulmonary venous obstruction is a severe urgent CHD, according to the CHD classification by by Slodki et al 11 The need of urgent intervention in this group of infants highlights the importance of prenatal diagnosis of TAPVC. Our experience so far had shown, that obstructed TAPVC associated with other heart abnormalities, despite good clinical condition of the newborns on first day of postnatal life, was a lethal condition on the following day 12 .…”

Great Vessels Anomalies – Prenatal Echocardiography and Neonatal Angio-CT – A Pictorial Essay

“…It helps parents and a medical team to plan a postnatal care. It is vital because some defects require treatment in first days of life and in other cases treatment can be postponed for 2 nd or 3 rd week of postnatal life or patient can be treated conservatively 3,4 .…”