Reversible cardiomyopathy in a patient with Marfan's syndrome. Case report.

Marfan´s syndrome is a multisystemic, autosomal dominant congenital abnormality of variable penetrance that affects the integrity of connective tissue. In the cardiovascular system, the dysfunction of the physiology of the aortic root and the myocardial fibrosis originates non-ischemic cardiomyopathy independent of valve lesions. Few data have been reported on the prevalence of arrhythmias and its impact on heart function. We present a 21-year-old man with Marfan's syndrome and heart failure with frequent supraventricular arrhythmias and aortic root dilation. After ablation in the posteroseptal area of the mitral ring and Tirone David Surgery, there was clinical improvement, the left ventricular ejection fraction increased dramatically from 33% to 46%, the left ventricular end-diastolic volume decreased from 90 ml/m2 to 77 ml/m2 and the NT-proBNP decrease from 1100 pg/mL at 180 pg/mL.

El síndrome de Marfan es una anomalía congénita multisistémica, autosómica dominante y de penetrancia variable que afecta a la integridad del tejido conectivo. En el sistema cardiovascular, también se ha descrito la disfunción de la fisiología de la raíz aórtica y la fibrosis miocárdica que origina una miocardiopatía no isquémica independiente de las lesiones valvulares. Se han comunicado pocos datos sobre la prevalencia de arritmias y su repercusión en la función cardiaca. Presentamos el caso de un varón de 21 años con síndrome de Marfan e insuficiencia cardiaca con frecuentes arritmias supraventriculares y dilatación de la raíz aórtica. Después de la ablación en la zona posteroseptal del anillo mitral y la cirugía de Tirone David, hubo mejoría clínica, la fracción de eyección ventricular izquierda aumentó espectacularmente del 33 al 46%, el volumen telediastólico ventricular izquierdo disminuyó de 90 a 77 mL/m* y el NT-proBNP disminuyó de 1100 a 180 pg/mL.

Assessment of Left Ventricular Myocardial Fibrosis in Adult Patients With Ebstein Anomaly: A Retrospective Cohort Study Based on Cardiac Magnetic Resonance and Histopathological Samples.

BACKGROUND: The association between Ebstein anomaly and myocardial fibrosis, particularly in the left ventricle, has been controversial. We aimed to assess the prevalence of replacement fibrosis with a focus on the left ventricle (LV) using cardiac magnetic resonance (CMR), make a histopathological association between LV fibrosis and CMR findings, and explore whether LV fibrosis is an independent risk factor for cardiovascular disease mortality using a derived risk score. METHODS: We performed a 12-year (2009-2021) retrospective cohort of adult patients with Ebstein anomaly who underwent CMR. The CMR evaluation included a comprehensive assessment of myocardial fibrosis by late gadolinium enhancement (LGE). Four postmortem samples were obtained from our cohort and stained using Masson trichrome to characterize LV fibrosis. We used Cox-regression analysis to identify and derive a prediction score that associated LV fibrosis with cardiovascular disease mortality. RESULTS: We included 57 adults with Ebstein anomaly (52% men; median age, 29.52 [interquartile range, 21.24-39.17] years), of whom 12 died during follow-up. LGE prevalence by CMR was observed in 52.6% in any chamber; LV-LGE in 29.8%. Histopathological findings revealed a mid-wall pattern with predominantly interstitial fibrosis and minimal replacement fibrosis. LV-LGE was associated with increased risk of cardiovascular disease mortality (hazard ratio, 6.02 [95% CI, 1.22-19.91]) attributable to lateral and mid-wall LV segment involvement. Our mortality score achieved an overall good prediction capacity (R2, 0.435; C statistic, 0.93; Dxy, 0.86). CONCLUSIONS: There is a high prevalence of LV fibrosis replacement in adults with Ebstein anomaly, characterized by specific CMR and histological patterns. Furthermore, LV-LGE fibrosis is an independent predictor of cardiovascular disease mortality, which could be integrated into risk assessment in clinical management.

Diagnosis, performance and added value of assessing ventricular dyssynchrony by phase analysis in patients with three-vessel disease: A single-center cross-sectional study in Mexico.

BACKGROUND: Three-vessel disease (3VD) is a cardiovascular disorder that affects the three main coronary arteries. Gated myocardial perfusion SPECT (GMPS) evaluates ventricular function, synchrony, and myocardial perfusion. However, the diagnostic performance of GMPS parameters to assess 3VD has not been fully explored. AIMS: To assess the univariate performance capacity of GMPS parameters, and to evaluate whether phase parameters could provide additional predictive value for the detection of patients with 3VD compared to control subjects. METHODS: We designed paired retrospective samples of GMPS images of patients with 3VD (stenosis > 70% of left anterior descending, right coronary, and circumflex coronary arteries) and without 3VD. A GMPS in rest-stress protocol was performed using 99mTc-Sestamibi and thallium and analyzed with the 3D method. Area under the receiver-operating characteristic curves (AUROC), decision curve analyses and diagnostic test performance were obtained for univariable analyses and stepwise binomial logistic regression for multivariable performance. RESULTS: 474 Patients were included: 237 with 3VD (84% males, mean age 61.7 ± 9.9 years) and 237 with normal GMPS (51% women, mean age 63.8 ± 10.6 years). The highest AUROC for perfusion parameters were recorded for SSS, SRS and TID. For dyssynchrony parameters, both entropy and bandwidth in rest and stress phases displayed the highest AUROC and diagnostic capacity to detect 3VD. A multivariate model with SRS ≥ 4, SDS ≥ 2, TID > 1.19 and sBW ≥ 48° displayed the highest diagnostic capacity (0.923 [95% CI 0.897-0.923]) to detect 3VD. CONCLUSION: Perfusion and dyssynchrony were the parameters which were most able to discriminate patients with 3VD from those who did not have CAD.

Computed tomography assessment of saw-tooth cardiomyopathy: a case series.

BACKGROUND: Saw-tooth cardiomyopathy (STC) is an unusual type of left ventricular dysplasia. To our knowledge, six cases have been reported in the literature. Two new cases are presented with a review of all the case reports that have been published. CASE SUMMARY: Two patients with STC were examined. The first one was a 69-year-old woman with shortness of breath on mild exertion and chest pain, and the second was a 49-year-old man with a history of myocardial infarction who required stent implantation and is now asymptomatic. Both patients revealed findings of STC in the cardiac computed tomography (CT). DISCUSSION: When analysing the cases and comparing them to the ones reported in the literature; STC is a generally benign heart disease, although the clinical spectrum can range from asymptomatic to heart failure. Imaging studies such as cardiac magnetic resonance and cardiac CT are essential for the diagnosis.

Case Report: A Rare Case of a Ventricular Perivascular Epithelioid Cell Tumor With Histologic Characteristics That Resembled a Primary Cardiac Rhabdomyoma.

We present the case of a young male patient with an initial diagnosis of a rhabdomyoma that was surgically treated at a different hospital when he was 17. After a 2-year disease-free period, the patient presented another intra-cardiac mass. He refused surgical treatment and died 5 years later. Post-mortem immunochemistry studies of both tumors led to the diagnosis of a primary malignant cardiac PEComa with histopathologic characteristics that resembled a rhabdomyoma with abundant "spider cells."

Assessment of Atypical Cardiovascular Risk Factors Using Single Photon Emission Computed Tomography in Mexican Women.

BACKGROUND: Ischemic heart disease (IHD) is a health care problem in women that increases morbimortality, particularly in developing countries. There is limited information regarding atypical risk factors associated with IHD in Mexican women. AIM: To explore risk factors in women that could contribute to IHD and myocardial dysfunction using the single photon emission computed tomography (SPECT) myocardial perfusion study (MPS). METHODS: We designed a cross-sectional study in which we evaluated atypical and typical risk factors using a clinical questionnaire. We performed a SPECT-MPS to evaluate the presence of ischemia/infarction, decreased left ventricular ejection fraction, systolic dyssynchrony and diastolic function by peak filling rate and time to peak filling rate. RESULTS: 172 women were included, 64 with IHD. Adverse events during pregnancy (premature birth and miscarriage), rheumatoid arthritis, gynecological conditions (menopause and age of first menstruation) and low educational level, together with previously known typical risk factors were associated with infarction or ischemia and ventricular dysfunction. Potential associated factors for systolic dyssynchrony were rheumatoid arthritis (OR: 2.90, 95% CI: 0.95-8.66, p = 0.054) and history of premature birth (OR: 0.13, 95% CI: 0.01-0.66, p <0.01). Although those women with arterial hypertension and smoking shown an increased risk for dyssynchrony, these factors were not statistically significant. Low-educational level (OR 2.16, 95% CI 1.1-4.18, p = 0.019) was associated with decreased peak filling rate. CONCLUSION: The presence of atypical risk factors in women could lead to decreased myocardial function, particularly in women at risk of developing IHD.

Left ventricular and atrial global strain evaluation within subtypes of ventricular remodeling.

BACKGROUND: Left ventricular hypertrophy is associated with poor prognosis and adverse events. Left ventricular and left atrial global strain and left atrial reservoir strain (LV-GS; LA-GS; LA-RS) could be used as markers for myocardial function in different ventricular remodeling forms. This study aimed to evaluate LV-GS and LA-GS scores in different ventricular remodeling variants and identify risk factors for myocardial dysfunction. METHODS AND RESULTS: This cross-sectional study was divided into four groups of ventricular remodeling: normal geometry, eccentric hypertrophy (EH), concentric hypertrophy (CH), and concentric remodeling (CR). Strain analysis was obtained using standardized protocols. We included 121 subjects, 33 with previous myocardial infarction (MI). We found that EH had the lowest LV-GS and CH, the lowest LA-GS, and LA-RS. Atrial and ventricular dysfunction was present in 40 (33%) and 14 (11.5%) subjects, respectively. Smoking, male sex, and previous MI were associated with LV dysfunction and smoking and dyslipidemia with LA dysfunction; EH was closely associated with LV dysfunction and CH with LA dysfunction. CONCLUSIONS: We conclude that different ventricular geometry types had echocardiographic profiles associated with different risk factors for dysfunction assessed by strain. The assessment of ventricular remodeling by global strain could be used as a complementary tool in the echocardiographic evaluation of ventricular and atrial function.

Optimal Medical Treatment vs. Invasive Approach in Patients with Significantly Obstructive Coronary Artery Disease and Ischemia.

INTRODUCTION: Stable ischemic heart disease (SIHD) is a condition that develops in subjects after myocardial infarction. Evidence suggests that optimal medical treatment (OMT) is not inferior to intervention (INT) using percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG). AIM: To compare clinical outcomes in subjects with SIHD who only received OMT and those who received INT+OMT. METHODS: We retrospectively examined subjects with SIHD who underwent myocardial perfusion study-SPECT/CT in a reference center in Mexico. We assigned two branches: INT+OMT (subjects with previous PCI or CABG) and OMT (subjects with antiplatelet drugs, ß-blockers, renin-angiotensin-system blockade, nitrates, calcium-channel blockers, and aggressive lipid-lowering therapy). Clinical outcomes at follow-up were angina relief, functional class improvement, hospitalization, myocardial reinfarction and death from any cause. RESULTS: We included 100 subjects; 51 with OMT and 49 with INT+OMT. 54 subjects had 1 affected vessel and 46 more than 2. INT+OMT group had up to 14 fold likelihood (95% CI: 3.38-63.35) of achieving angina relief and 2.2 fold likelihood (95% CI: 0.92-5.57, p = 0.077) for functional class improvement. No differences were found in hospitalization, myocardial infarction and death from any cause compared to OMT. CONCLUSIONS: Subjects with OMT have no higher risk of adverse clinical outcomes compared to INT+OMT. However, the INT+OMT provides angina relief and functional class improvement compared to OMT.

Echocardiographic correlates of severe pulmonary hypertension in adult patients with ostium secundum atrial septal defect.

OBJECTIVE: To identify echocardiographic factors that correlate with pulmonary hypertension (PH) in adults with ostium secundum atrial septal defect (ASD). METHODS: Between November 2009 and November 2013, 92 adults with ASD were studied. All had clinical history and transthoracic echocardiogram. RESULTS: Thirty-nine percent of patients had severe PH defined as systolic pulmonary artery pressure (sPAP) of 70 mm Hg or more. The size of ASD (31.84±8.21 mm) and a right-sided tricuspid inflow E-wave to tissue Doppler e'-wave ratio >6.2 correlated with severe PH with AUC of 0.704 (CI 95%=0.59 to 0.818, P<.001) and 0.65 (CI 95%=0.531 to 0.773, P<.014), respectively. Multivariate logistic regression showed that sPAP >70 mm Hg was the variable that most precisely correlated with right ventricular (RV) dysfunction as evidenced by TAPSE <17 mm and RV fractional shortening area (RVFSA) <35%. Left ventricular (LV) diastolic function was also significantly reduced in the group with severe PH with mitral inflow E/A ratio of 0.73±0.23 vs 1.13±0.42 in the group without severe PH (sPAP <70 mm Hg, (P=.001). The pulmonary (Qp) to systemic (Qs) cardiac output ratio (3.09±1.12) and right-sided tissue Doppler S <9.5 cm/s most accurately predicted a Tei index >0.55. CONCLUSIONS: Larger size of ASD using the QP/QS ratio and increased right-sided tricuspid E/e' ratio correlated with severe PH with a sPAP of 70 mm Hg or more. Patients with severe PH had more severe RV dysfunction as evaluated by TAPSE and RVFSA in comparison to those with PH <70 mm Hg. LV diastolic function was also reduced in the severe PH group.

Prevalence of Congenital Heart Disease and Pulmonary Hypertension in Down's Syndrome: An Echocardiographic Study.

BACKGROUND: Down's syndrome (DS) is a genetic anomaly, which undergoes increased morbidity and mortality when associated with congenital heart disease (CHD). The aims of the study were to determine the prevalence of CHD and pulmonary hypertension (PH) in DS. METHODS: One hundred twenty-seven patients with DS living in Mexico City were evaluated by physical exam, electrocardiogram and echocardiogram. RESULTS: CHD was found in 40%. In 80% (n = 102) PH was present [systolic pulmonary artery pressure (SPAP) of 47 ± 19 mm Hg and mean pulmonary artery pressure (MPAP) of 32 ± 11 mm Hg]. Patients with CHD and PH were classified as having 1) no shunt (n = 18) with SPAP of 37 ± 9 mm Hg and MPAP of 25 ± 6 mm Hg and 2) with shunt (n = 26) with PASP of 57 ± 29 mm Hg and MPAP of 38 ± 19 mm Hg (p ≤ 0.001). In those without CHD or with CHD without shunt (n = 76), SPAP was 37 ± 19 mm Hg and the MPAP 25 ± 6 mm Hg. The prevalence of PH in DS was 5.9% at one year and 15% at 10 years. The odds ratio of PH in DS with CHD was 7.3 vs. 3 without CHD. CONCLUSION: DS has a high prevalence of CHD and PH. PH prevalence increases when it is associated with CHD. The pathophysiology of PH in DS without CHD should be studied in the near future. Echocardiography is an indispensible tool for evaluation of DS.

Understanding atrioventricular septal defect: anatomoechocardiographic correlation.

OBJECTIVE: Correlate the anatomic features of atrioventricular septal defect with echocardiographic images. MATERIALS AND METHODS: Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation. RESULTS: Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia. CONCLUSION: Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography.

Ebstein's Anomaly: anatomo-echocardiographic correlation.

OBJECTIVE: The aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) the right ventricle (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images. METHODS: Thirty hearts with EA were studied. The alterations of each portions of the RV were described. Fifty adult patients with this anomaly were studied by echocardiography. RESULTS: Anatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of these 2 had transposition of the great arteries (TGA) and one double outlet right ventricle (DORV). The degree of tricuspid valve (TV) displacement showed a spectrum from I to III. The inlet of the RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in 5. In 25 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of these last patients 5 had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 45. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found. CONCLUSION: The EA affects the whole RV and the anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, therapeutic decisions and prognosis.

Aortic obstruction: anatomy and echocardiography.

Echocardiography is a valuable non-invasive technique for identifying the site and type of aortic obstruction. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to correlate the echocardiographic images with anatomic specimens of equivalent valvular and supravalvular aortic obstruction. Specimens were part of the collection of the Department of Embryology. Fifty six patients were studied, and forty specimens with aortic obstruction were analyzed. Echocardiographic characteristics: Thirty one (55.3%) patients were women and twenty five (44.7%) men. Valvular aortic obstruction was found in Thirty six patients (64.3 %) and supravalvular aortic obstruction in twenty (35.7%). Anatomic characteristics: Of the forty specimens examined, twenty one (52.5%) had valvular aortic obstruction and nineteen (47.5%) supravalvular aortic obstruction. The anatomoechocardiographic correlation clearly showed that the anatomic findings of the specimen hearts and aortas corresponded to echocardiographic images of valvular and supravalvular aortic obstruction and provided solid corroboration of echocardiographic diagnoses.

Non-compacted cardiomyopathy: clinical-echocardiographic study.

UNLABELLED: The aim of the present study was to describe the clinical and echocardiographic findings of ventricular noncompaction in adult patients. Fifty-three patients underwent complete clinical history, electrocardiogram, Holter and transthoracic echocardiogram. Forty patients (75%) were in class I/II of the New York Heart Association, and 13 (25%) in class III/IV. Ventricular and supraventricular escape beats were found in 40% and 26.4%, respectively. Holter showed premature ventricular contractions in 32% and sustained ventricular tachycardia in 7.5%. Ventricular noncompaction was an isolated finding in 74% of cases and was associated with other congenital heart disease in 26%. Noncompacted ventricular myocardium involved only left ventricle in 62% of the patients and both ventricles in 38%. The mean ratio of noncompacted to compacted myocardial layers at the site of maximal wall thickness was 3.4 +/- 0.87 mm (range 2.2-7.5). The presence of ventricular noncompaction in more than three segments was associated with a functional class greater than II and ventricular arrhythmia with demonstrable statistical significance by chi2(p < 0.003). CONCLUSION: a) Noncompacted cardiomyopathy is a congenital pathological entity that can occur in isolated form or associated with other heart disease and often involves both ventricles. b) A ratio of noncompacted to compacted myocardium greater than 3 and involvement of three or more segments are indicators of poor prognosis. c) Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular noncompaction and establish prognostic factors.

Atrial septal defect: anatomoechocardiographic correlation.

This study was undertaken to enhance understanding of the anatomic abnormalities involved in atrial septal defect (ASD) based on the anatomoechocardiographic comparison of equivalent specimens of ASD and the echocardiograms of patients with this anomaly. Of the 72 heart specimens, two had common atrioventricular canal (2.8%) and one had absence of right atrioventricular connection (1.4%). In all, 46 (63.8%) had fossa ovalis type ASD, two (2.8%) had true ostium secundum type ASD, two (2.8%) had ostium primum type ASD, two (2.8%) had superior sinus venosus type ASD, and two (2.8%) had inferior sinus venosus type ASD. One (1.4%) specimen had a coronary venous sinus type ASD and 14 (19.4%) had mixed type ASD. Of the 144 patients evaluated with echocardiography 15 (10.4%) had common atrioventricular canal, one (0.7%) had right absence of atrioventricular connection, 105 (72.9%) had ostium secundum type ASD (fossa ovalis), 10 (7%) had mixed type ASD, 9 (6.2%) had superior venous sinus type ASD, two (1.4%) had ostium primum type ASD, and two (1.4%) had true fossa ovalis type ASD. This series leads us to conclude that the key to successful management of ASD depends on understanding echocardiographic images in terms of anatomic specimens to provide appropriate evaluations for therapeutic decisions and establishment of prognoses.

Understanding left ventricular outflow obstruction: anatomoechocardiographic correlation.

OBJECTIVE: Echocardiography is a valuable noninvasive technique for identifying the site and type of obstruction in the left ventricular outflow tract. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. METHODS: This study was undertaken to provide improved understanding of the anatomy of left ventricular outflow obstruction with corresponding side-by-side echocardiographic images. Specimens were part of the collection of the Department of Embryology of the Instituto Nacional de Cardiología "Ignacio Chávez," Mexico City, Mexico. Twenty-eight patients were studied by echocardiography, and 22 specimens with different types of left ventricular outflow tract obstruction were analyzed. RESULTS: Echocardiographic characteristics: Eighteen (64.3%) patients were women and 10 (35.7%) men. Three had fibrous rings, 19 with obstructive asymmetric septal hypertrophic cardiomyopathy, 1 with posterior displacement of the infundibular septum, 2 with accessory mitral valve tissue, 2 with anomalous insertion of mitral valve chordae tendineae, and 1 with malposition of the mitral valve and papillary muscles. Anatomic characteristics: Twelve had fibrous ring, 2 asymmetric septal hypertrophic cardiomyopathy, 2 posterior displacement of the infundibular septum, 1 ectopic mitral tissue, 1 anomalous insertion of mitral valve chordae tendineae in the ventricular septum, 3 malposition of the mitral valve and papillary muscles, and 1 subaortic infundibulum. CONCLUSION: The anatomoechocardiographic correlation clearly demonstrates the mechanism of subaortic obstruction. The comparison with corresponding echocardiographic images of different subaortic obstruction types is potentially quite valuable in enhancing the echocardiographer's understanding especially of complex subaortic obstruction.

Echocardiometric evaluation of cardiovascular abnormalities in Marfan syndrome.

UNLABELLED: Marfan syndrome is an inherited disorder of connective tissue with manifestations in various organ-systems including cardiovascular system. The aim of this study was to characterize and determine the frequency of cardiovascular alterations by echocardiography in 2 age cohorts of Mexican patients with Marfan syndrome and their comparisons with control groups. MATERIAL AND METHODS: Sixty six with Marfan syndrome and 33 control patients were evaluated by echocardiography. Segments of the aorta and pulmonary artery were measured at different levels, cardiac valves were examined for prolapse and the interatrial septum was assessed for septal aneurysm. Numeric values were corrected for the body surface area and compared with the control group. RESULTS: Mean significant values between group I (children) and Group II (adults) were as follows: aortic annulus 16.62 +/- 4.57 mm/m2 vs 12.81 +/- 1.95 (p < 0.001), aortic root 23.30 +/- 7.49 mm/m2 vs 18.36 +/- 2.97 (p < 0.001), sinuses of Valsalva 24.14 +/- 7.29 mm/m2 vs 19.84 +/- 3.59 (p < 0.001), ascending aorta 18.43 +/- 5.90 mm/m2 vs 17.02 +/- 4.79 (p < 0.001), aortic arch 16.12 +/- 4.73 mm/m2 vs 14.20 +/- 2.68 (p < 0.001). Pulmonary valve prolapse was seen in 10/22 (45.5%) vs 7/44 (15.9%), p < or = 0.03. Interatrial septal aneurysm was found in 3/22 (13.6%) vs 20/44 (45.5%), p < or = 0.03. There was a significative diference in the presence of atrial septal aneurysm between the adult group and control group (p < 0.001). CONCLUSIONS: The incidence of cardiovascular abnormalities in our series is similar to that in the literature with the exception of the very high incidence of pulmonary valve prolapse vs control groups, then it suggests that the clinical manifestations of MFS are strikingly severe in the Mexican population. Also a high incidence of interatrial septal aneurysm (34.9%) in comparison to control groups (18.2%) was found.