The Fontan Operation
References First author Journal Year Design n Age BMI Male (%) Diagnosis APC/TCPC NYHA Peak VO2 ACEI/ARB ß Diuretics [24] Myers KA JASE 2013 Fontan 22 14.9 (7.6–18.7) 19.5 (15.0–25.7)…
Pregnancy and Delivery
Fig. 9.1 Incidence of dissection of the aorta in Marfan syndrome. Marfan syndrome patients tend to experience dissection of the aorta during pregnancy than nonpregnant period (4.4 % vs. 0.9 %, p < 0.05)…
Aortic Coarctation
Fig. 16.1 Schematic depiction of the three possible locations of the infolding relative to the patent ductus arteriosus (PDA). (a) Pre-ductal coarctation. (b) Para-ductal coarctation. (c) Post-ductal coarctation 16.2.2 Epidemiology…
The Marfan Syndrome
Family history Aortic dilation (Z ≥ 2) or dissection Ectopia lentis Systemic score (≥7 of 20) Pathogenic FBN1 mutation X X X X X X X Xao X X X X X …
Aortopathies: Clinical Manifestation
General Advanced age Arteriosclerosis Arterial hypertension Genetic aortic syndromes (monogenetic diseases) Nonsyndromic Familial thoracic aortic aneurysm syndrome caused by mutations in the following genes ACTA2 MYH11 MMP3 MMP9 SMAD3 Syndromic…
Genetics of Marfan Syndrome, Related Disorders, and Bicuspid Aortic Valve
General criteria Condition In the absence of family history (1) Ao (Z≥2) and EL = MFS (2) Ao (Z≥2) and FBN1 = MFS (3) Ao (Z≥2) and Syst (≥7pts) =…
Hypoplastic Left Heart Syndrome
Fig. 18.1 Progressive neo-aortic dilation in patients with hypoplastic left heart syndrome. Box plots depicting the median value (middle line of box), the 25th and 75th percentiles (upper and low…
Future Research Projects on Aortopathy in Congenital Heart Anomalies
Fig. 21.1 The time schedule for “TRANSIT” study 21.2.4 Outcome and Clinical Significance The primary outcome is the time-dependent change in aortic root diameter, specifically, the enlargement rate per year…
Bicuspid Aortic Valve
Type of study Frequency of BAV in the general population Frequency of aortopathy in BAV Autopsy 0.5–1.39% (meta-analysis of necropsy studies) [4] 7.5–11.2% of fatal aortic dissections have BAV [5–7]…
Medications
© Springer Japan KK 2017Koichiro Niwa and Harald Kaemmerer (eds.)Aortopathy10.1007/978-4-431-56071-5_10 10. Medications Hsin-Hui Chiu1, 2 and Mei-Hwan Wu1 (1) Department of Pediatrics, National Taiwan University Hospital and School of Medicine, College of Medicine, National Taiwan University, No….
