“Functionally” Univentricular Hearts: Impact of Pre-Natal Diagnosis

Abstract

Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, particularly for the identification of complex malformation. "Functionally" univentricular hearts categorize a huge variety of heart malformations. Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of "univentricular" heart. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in "functionally" univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment.

Ethical issues after pre-natal diagnosis

“…A f t e r f e t a l d i a g n o s i s o f u n i v e n t r i c u l a r h e a r t (4)(5)(6)8,14,15,19,21,22,24,25,28,39), the decisionmaking process has been oriented towards for pregnancy termination or compassionate care in 8% of cases (19), through 16% (5,6), 30 (14,15), 33% (4), reaching 47% (28), due to one or more of the following reasons: low hospital survival, impaired quality of life, child suffering (8,14), stress for the entire family, including siblings (8,24), association of genetic abnormalities and/or severe non-cardiac defects (28).…”

“…After fetal diagnosis of complex congenital heart defect a comprehensive multi-specialties pre-natal screening, including chromosomes assessment, complete intraand extra-cardiac evaluation, to provide the family with an appropriate explanation of the expected future (5)(6)(7)(14)(15)(16)(17)19,21,24,29,39). The parents and their families have the right to receive all data useful to prepare and support them (24).…”

“…Following pre-natal diagnosis of congenital heart defect parents and family face a dramatic psychological crisis because of their state of shock, contradictory information available on potential outcomes, limited availability of time for decisions and for autonomous choices (8,39). Furthermore, not all the parents wish to perform autonomous choices due to family or other societal pressures (8).…”

“…Counselling the parents, already complicated by the communications between healthcare providers and families, can present additional difficulties due to education, cultural and religious background, individual cognitive and emotional processes (9,10,24,26,39). Cross-cultural patient care is a challenging issue for the caregivers: dealing with families rejecting medical treatments because of their educational and/or religious backgrounds requires an open mind to successfully propose a medical plan (10,26,39).…”

Editorial: Univentricular Heart

“…The patients with “functionally” univentricular heart have been taken into consideration from the moment of pre-natal diagnosis ( 34 ) through detailed analysis of the complex and variable underlying morphology ( 35 ).…”