Neues aus der Kinderkardiologie
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“Recurrent Congenital Heart Diseases among Neonates Born to Mothers with Congenital Heart Diseases”
3 - fold increased risk of CHD in children of mothers with CHD
Title: “Recurrent Congenital Heart Diseases among Neonates Born to Mothers with Congenital Heart Diseases”
Citation:T. Yokouchi‑Konishi, J. Yoshimatsu, M. Sawada, T. Shionoiri, A. Nakanishi, C Horiuchi, et al., Recurrent Congenital Heart Diseases Among Neonates Born to Mothers with Congenital Heart Diseases, Pediatric Cardiology (2019) 40:865–870.
Plain text summary:Dr. univ. (I), Alessia Callegari; PD Dr. Christian Balmer, Pediatric Cardiology Unit, Children’s Hospital Zurich
Text:In the general population, the incidence of congenital heart disease (CHD) is known to be circa 1%, but is reported to be as high as 7% in neonates of mothers with CHD. This has a consistent clinical impact, especially considering the growing population of adult women with CHD.
This single centre retrospective analysis et al. reports on the recurrence rate of CHD in case of maternal CHD in Japan. For study purposes, neonatal CHD was defined as CHD echocardiographic diagnosed within 1 month of age.
Out of 803 neonates (10 twins) from 529 women with CHD a total of 25 neonates was affected by CHD. The estimated average rate of CHD recurrence was therefore 3.1%, meaning that the risk of CHD was 3 times greater than that of mothers with healthy hearts. Almost half of neonates with CHD had the same phenotype as their mother: especially right obstructive lesions showed a high recurrence rate. Lastly, they reported a three- to ninefold greater risk of CHD in case of siblings with CHD.
Even interpreted with appropriate understanding of the associated limitations, these results remark the need of an appropriate preconception counseling in women with CHD, considering the higher risk of recurrence. In order to gain a proper insight on the risk associated to the different type of CHD a larger and prospective study is needed.
“The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data”
Title: “The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data”
Citation: Hormaza VM, Conaway M, Schneider DS, Vergales JE. The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data. Congenital Heart Disease. 2019;14:274–279.
Plain text summary: Dr. Sebastiano A.G. Lava, PD Dr. Stefano Di Bernardo; Pediatric Cardiology Unit, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne
Hormaza and colleagues retrospectively looked at the right ventricular function prior to stage 2 univentricular palliation (Glenn procedure), by exploting the freely available NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial data set. They performed a case-control study, by comparing 265 patients doing well after the Glenn procedure (but before progressing to the stage 3, Fontan completion) with 18 patients with a “failing stage 2”, that was defined as death, patient transplanted or listed for transplant.
They found that, prior to stage 2, future “failing Glenn” had an inferior right ventricular fractional area change as well as superior right ventricular volumes, both end-diastolic and end-systolic, as compared to patients doing well after stage 2 palliation. They also found that every 10% increase in right ventricular ejection fraction was associated with a 63% decrease in length of hospital stay and 68% decrease in duration of intubation.
Notwithstanding the several limitations of this study and acknowledging the retrospective study design precluding any conclusion on cause-effect relationships, this study suggests that a lower right ventricular function prior to stage 2 palliation might be associated with an increased risk of failing stage 2 later on. Definition of cutoff values and, thus, timely identification of these patients might help in proactively improving their management.