PMID- 20849479 OWN - NLM STAT- MEDLINE DCOM- 20110208 LR - 20161125 IS - 1540-8175 (Electronic) IS - 0742-2822 (Linking) VI - 27 IP - 8 DP - 2010 Sep TI - Echocardiographic study of a Mestizo-Mexican population with Marfan syndrome. PG - 923-30 LID - 10.1111/j.1540-8175.2010.01208.x [doi] AB - BACKGROUND: The cardiovascular manifestations of Marfan syndrome (MFS) are the main causes of morbidity and mortality. This study describes the clinical and echocardiographic findings in a Mestizo-Mexican population affected by the disease. METHODS: A total of 166 patients previously diagnosed with MFS were recruited for the study, 114 of them underwent complete clinical history, with emphasis on Ghent nosology criteria, and transthoracic echocardiography, with 68 patients also undergoing transesophageal study. RESULTS: Major cardiovascular criteria from the Ghent nosology predominated in adults (P < 0.0001), minor criteria in children (P = 0.007). Among pediatric patients, 83% had a New York Heart Association (NYHA) functional class of I; however, 64% of the adult patients had an NYHA class >/=II, (P < 0.0001). Corrected aortic echocardiographic measurements of both groups demonstrated statistically significant differences. Children had a greater prevalence of mitral valve prolapse, while adults more frequently presented with aortic complications. Seven patients died during follow-up from aortic complications, one child and six adults. CONCLUSIONS: Based on the data, we can conclude that MFS in the Mestizo-Mexican population has a distinctly different clinical pattern in children and adults, and a graver prognosis in adults. Adult patients with MFS are significantly more likely, than children, to have aortic dilation, aortic aneurysm, aortic regurgitation, aneurysm rupture, aortic dissection, and fatal outcome. Children with MFS are more likely, than adults, to present with asymptomatic mitral and tricuspid prolapse and mitral valve regurgitation. CI - (c) 2010, Wiley Periodicals, Inc. FAU - Espinola-Zavaleta, Nilda AU - Espinola-Zavaleta N AD - Immunology Department, Instituto Nacional de Cardiologia Ignacio Chavez, Juan Badiano No. 1, Mexico City, Mexico. FAU - Iqbal, Fahad M AU - Iqbal FM FAU - Nanda, Navin C AU - Nanda NC FAU - Enriquez-Rodriguez, Eduardo AU - Enriquez-Rodriguez E FAU - Amezcua-Guerra, Luis M AU - Amezcua-Guerra LM FAU - Bojalil-Parra, Rafael AU - Bojalil-Parra R FAU - Reyes, Pedro A AU - Reyes PA FAU - Soto, Maria Elena AU - Soto ME LA - eng PT - Journal Article PL - United States TA - Echocardiography JT - Echocardiography (Mount Kisco, N.Y.) JID - 8511187 SB - IM MH - Adult MH - Child MH - Female MH - Humans MH - Male MH - Marfan Syndrome/*diagnostic imaging/*epidemiology MH - Mexico/epidemiology MH - Prevalence MH - Ultrasonography/*statistics & numerical data EDAT- 2010/09/21 06:00 MHDA- 2011/02/09 06:00 CRDT- 2010/09/21 06:00 PHST- 2010/09/21 06:00 [entrez] PHST- 2010/09/21 06:00 [pubmed] PHST- 2011/02/09 06:00 [medline] AID - 10.1111/j.1540-8175.2010.01208.x [doi] PST - ppublish SO - Echocardiography. 2010 Sep;27(8):923-30. doi: 10.1111/j.1540-8175.2010.01208.x.