BACKGROUND: Left ventricular noncompaction (LVNC) has rarely been described in the fetus. METHODS: The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment. RESULTS: Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035). CONCLUSIONS: Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.
BACKGROUND: Left ventricular noncompaction (LVNC) has rarely been described in the fetus. METHODS: The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment. RESULTS: Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035). CONCLUSIONS: Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.
Authors: Claudia Stöllberger; Christian Wegner; Abraham Benatar; Thomas K Chin; Joanna Dangel; Danielle Majoor-Krakauer; Tapas K Mondal; Shanthi Sivanandam; Norman H Silverman; Jaap van Waning; Josef Finsterer Journal: Pediatr Cardiol Date: 2016-03-31 Impact factor: 1.655
Authors: Francesco Negri; Antonio De Luca; Enrico Fabris; Renata Korcova; Carlo Cernetti; Chrysanthos Grigoratos; Giovanni Donato Aquaro; Gaetano Nucifora; Paolo G Camici; Gianfranco Sinagra Journal: Heart Fail Rev Date: 2019-05 Impact factor: 4.214
Authors: Yasuhiro Kosaka; Katarzyna A Cieslik; Ling Li; George Lezin; Colin T Maguire; Yukio Saijoh; Kazuhito Toyo-oka; Michael J Gambello; Matteo Vatta; Anthony Wynshaw-Boris; Antonio Baldini; H Joseph Yost; Luca Brunelli Journal: Mol Cell Biol Date: 2012-10-15 Impact factor: 4.272