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Wayne H Franklin - One of the best experts on this subject based on the ideXlab platform.

  • dual chamber epicardial pacing in neonates with congenital heart block
    The Journal of Thoracic and Cardiovascular Surgery, 2007
    Co-Authors: Robert D Stewart, Angela M Kelle, Carl L Backer, Sabrina Tsao, Wayne H Franklin
    Abstract:

    Objective This review evaluates the outcome of a treatment strategy using dual-chamber pacemakers for neonates with congenital heart block. Methods From 1989 to 2006, 10 neonates had dual-chamber epicardial pacemaker placement for congenital heart block. Mean age and weight were 4.4 ± 5.6 days and 2.8 ± 0.6 kg. Four patients had Heterotaxy Syndrome and required concomitant cardiac procedures. One patient had fetal hydrops, myocarditis, and cardiomyopathy. Five patients had structurally normal hearts. Sternotomy (2 partial, 8 full) and unipolar leads (2 non–steroid eluting, 18 steroid-eluting) were used in all. Generators were placed in submuscular bilateral rectus sheath pockets. Results Successful atrioventricular synchrony was established in all patients. Mean P-wave sensing was 4.7 ± 2.6 mV and atrial voltage threshold was 0.8 ± 0.3 V. Mean R-wave sensing was 13.0 ± 5.7 mV and ventricular voltage threshold 0.9 ± 0.5 V. There were no wound complications or acute lead failures. Median postoperative stay was 14 days. Three of the 4 patients with Heterotaxy died at 3 days, 14 days, and 15 months postoperatively. The patient with cardiomyopathy died suddenly at 6 months of acute myocarditis. No patient with a structurally normal heart died ( P Conclusions Implantation of a dual-chamber epicardial pacemaker in neonates with congenital heart block is technically feasible and results in excellent outcomes in patients with structurally normal hearts. System longevity at 6 years is excellent. Patients with congenital heart block and Heterotaxy Syndrome have a poor prognosis despite dual-chamber pacing.

Angela M Kelle - One of the best experts on this subject based on the ideXlab platform.

  • dual chamber epicardial pacing in neonates with congenital heart block
    The Journal of Thoracic and Cardiovascular Surgery, 2007
    Co-Authors: Robert D Stewart, Angela M Kelle, Carl L Backer, Sabrina Tsao, Wayne H Franklin
    Abstract:

    Objective This review evaluates the outcome of a treatment strategy using dual-chamber pacemakers for neonates with congenital heart block. Methods From 1989 to 2006, 10 neonates had dual-chamber epicardial pacemaker placement for congenital heart block. Mean age and weight were 4.4 ± 5.6 days and 2.8 ± 0.6 kg. Four patients had Heterotaxy Syndrome and required concomitant cardiac procedures. One patient had fetal hydrops, myocarditis, and cardiomyopathy. Five patients had structurally normal hearts. Sternotomy (2 partial, 8 full) and unipolar leads (2 non–steroid eluting, 18 steroid-eluting) were used in all. Generators were placed in submuscular bilateral rectus sheath pockets. Results Successful atrioventricular synchrony was established in all patients. Mean P-wave sensing was 4.7 ± 2.6 mV and atrial voltage threshold was 0.8 ± 0.3 V. Mean R-wave sensing was 13.0 ± 5.7 mV and ventricular voltage threshold 0.9 ± 0.5 V. There were no wound complications or acute lead failures. Median postoperative stay was 14 days. Three of the 4 patients with Heterotaxy died at 3 days, 14 days, and 15 months postoperatively. The patient with cardiomyopathy died suddenly at 6 months of acute myocarditis. No patient with a structurally normal heart died ( P Conclusions Implantation of a dual-chamber epicardial pacemaker in neonates with congenital heart block is technically feasible and results in excellent outcomes in patients with structurally normal hearts. System longevity at 6 years is excellent. Patients with congenital heart block and Heterotaxy Syndrome have a poor prognosis despite dual-chamber pacing.

Lauri A. Aaltonen - One of the best experts on this subject based on the ideXlab platform.

  • Recessively Inherited Right Atrial Isomerism Caused by Mutations in Growth/Differentiation Factor 1 (GDF1)
    2016
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen, Rainer Lehtonen
    Abstract:

    g.oxfordjournals.org/ D ow nloaded from 2 Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbancies in the left-right axis development resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate-gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidenc

  • recessively inherited right atrial isomerism caused by mutations in growth differentiation factor 1 gdf1
    Human Molecular Genetics, 2010
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Auli Karhu, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen
    Abstract:

    Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbances in the left-right axis development, resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal-recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidence that RAI can occur as a recessively inherited condition, with GDF1 as the culprit gene. The results will shed light on the biological basis of human laterality defects and facilitate molecular diagnosis of RAI.

Eero Kajantie - One of the best experts on this subject based on the ideXlab platform.

  • Recessively Inherited Right Atrial Isomerism Caused by Mutations in Growth/Differentiation Factor 1 (GDF1)
    2016
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen, Rainer Lehtonen
    Abstract:

    g.oxfordjournals.org/ D ow nloaded from 2 Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbancies in the left-right axis development resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate-gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidenc

  • recessively inherited right atrial isomerism caused by mutations in growth differentiation factor 1 gdf1
    Human Molecular Genetics, 2010
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Auli Karhu, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen
    Abstract:

    Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbances in the left-right axis development, resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal-recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidence that RAI can occur as a recessively inherited condition, with GDF1 as the culprit gene. The results will shed light on the biological basis of human laterality defects and facilitate molecular diagnosis of RAI.

Kristiina Aittomäki - One of the best experts on this subject based on the ideXlab platform.

  • Recessively Inherited Right Atrial Isomerism Caused by Mutations in Growth/Differentiation Factor 1 (GDF1)
    2016
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen, Rainer Lehtonen
    Abstract:

    g.oxfordjournals.org/ D ow nloaded from 2 Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbancies in the left-right axis development resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate-gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidenc

  • recessively inherited right atrial isomerism caused by mutations in growth differentiation factor 1 gdf1
    Human Molecular Genetics, 2010
    Co-Authors: Eevi Kaasinen, Kristiina Aittomäki, Marianne Eronen, Pia Vahteristo, Auli Karhu, Jukka-pekka Mecklin, Eero Kajantie, Lauri A. Aaltonen
    Abstract:

    Right atrial isomerism (RAI) is a Heterotaxy Syndrome with disturbances in the left-right axis development, resulting in complex heart malformations and abnormal lateralization of other thoracic and abdominal organs. Although autosomal-recessive inheritance of Heterotaxy Syndrome is seen in multiple families, underlying gene defects have remained unknown. Here we identify the molecular genetic basis of a kindred with five siblings with RAI. Linkage analysis and positional candidate gene approach showed that the affected children were compound heterozygotes for truncating mutations in the growth/differentiation factor 1 (GDF1) gene. Individuals heterozygous for the mutations were clinically healthy. This finding, supported by the similar phenotype in Gdf1 knockout mouse, provides firm evidence that RAI can occur as a recessively inherited condition, with GDF1 as the culprit gene. The results will shed light on the biological basis of human laterality defects and facilitate molecular diagnosis of RAI.