International Fetal Cardiac Intervention Registry

Abstract

We describe the contents of the IFCIR and present post-natal data to suggest potential benefit to fetal therapy among pregnancies considered for possible intervention and support proposals for additional work.

Immediate effects and outcome of in‐utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

“…The International Fetal Cardiac Intervention Registry reported 16 fetal pulmonary valvuloplasties, of which 11 procedures were successful and resulted in seven liveborn patients, five of which were discharged with a biventricular circulation. However, no data on preprocedure RV dimensions or postprocedure RV growth were recorded in these reports13.…”

“…Therefore, prenatal treatment could be a unique opportunity to take advantage of a better ventricular growth than that achievable postnatally. A few case reports and small case series have shown that fetal pulmonary valvuloplasty is technically feasible and associated with continued growth of RV structures8, 9, 10, 11, 12, 13. The International Fetal Cardiac Intervention Registry reported 16 fetal pulmonary valvuloplasties, of which 11 procedures were successful and resulted in seven liveborn patients, five of which were discharged with a biventricular circulation.…”

Trends in Congenital Heart Disease

“…16,17 The development of fetal echocardiography has provided a window into the evolution of CHD in utero, and fetal intervention, the possibility to potentially alter its course. 18,19 The pharmacology of cardiovascular medications has been more rigorously studied in young children and in those with Fontan physiology, and clinical trials to obtain pediatric labeling have been promoted through the exclusivity extension provision under the Food and Drug Administration Modernization Act. 20 Concurrent with these advances, we have seen the training and clinical maturation of a generation of physicians and allied healthcare practitioners, who have disseminated the knowledge and skills needed to treat pediatric and CHD globally.…”

Reference Ranges for the Size of the Fetal Cardiac Outflow Tracts From 13 to 36 Weeks Gestation

“…The reference ranges for cardiac outflow tracts and z scores presented in this study can be applied in the assessment of patients with cardiac asymmetry, pulmonary stenosis, right ventricular outflow tract obstruction, or aortic stenosis 2,3,5,10,11,13,15,16,18,[24][25][26][27][28][29][30][31][32] ; in these cases knowledge of the accurate z score can aid in predicting the need for neonatal intervention and to plan perinatal management. Z scores may also be useful in forecasting the natural history of valve stenosis and in designing clinical trials on the value of in utero valvuloplasty, where patients can be stratified according to z scores.…”

“…8 The impact of this method of assessment is well described in fetal cardiology particularly with respect to prediction of postnatal development of coarctation of the aorta, early neonatal intervention in tetralogy of Fallot and of single versus biventricular repair in cases of critical left or right ventricular outflow tract obstruction, 5,6,[9][10][11][12][13][14][15] and identification of suitable cases for fetal cardiac intervention. [16][17][18] Reference ranges of fetal outflow tracts have been published previously, but the number of cases examined was small ranging from 130 to 390. [19][20][21] Lee at al 22 published a series of 2735 cases, but measurements of the aortic and ductal arches were not reported.…”