PURPOSE: The gap in evidence in the management of multivalvular lesions can be addressed by providing more data on clinical and echocardiographic outcomes after percutaneous transmitral commissurotomy (PTMC) in patients with concomitant significant tricuspid regurgitation (TR) at baseline. METHODS This is a single-center cohort study of adult Filipinos 19 years or older, admitted between January 2019 and October 2020 due to severe mitral stenosis with moderate to severe TR subjected to PTMC. The outcome post-PTMC was divided into 2 groups: significant TR, which included the progression of moderate to severe TR or persistence of severe TR, and insignificant TR group, which included those with mild TR, regression of moderate to mild TR, severe to moderate, or persistence of moderate TR. The clinical and echocardiographic parameters of these groups were compared at baseline, at 24 hours, 1 month, and 6 months postprocedure. The numerical data between significant and nonsignificant TR were compared using nonparametric Mann–Whitney U test and categorical data using the x2 test.
Rheumatic Heart Disease ; Mitral Valve Stenosis ; Tricuspid Valve Insufficiency ; tricuspid regurgitation

PURPOSE

The combined mitral stenosis (MS) and aortic regurgitation (AR) impose opposite loading conditions on the left ventricle. Physiologically, the relief of MS may increase the severity of AR.

METHODS

Participants were Filipinos 19 years or older, admitted because of severe MS with mild to moderate AR. The outcome of percutaneous transmitral commissurotomy (PTMC) was divided into two groups: (1) significant AR that included the increase in severity of AR: mild to moderate/severe or moderate to severe AR, and (2) the insignificant AR group, which included any decrease in the severity of AR: moderate to mild or persistence of mild or moderate AR. These groups were compared from baseline, 24 hours, 1 month, and 6 months using the same echocardiographic parameters. The numerical data between significant and insignificant tricuspid regurgitation were compared using nonparametric Mann-Whitney U test and categorical data using the χ2 test.

RESULTS

A total of 43 participants were analyzed. At 24 hours post-PTMC, the significant AR group had significantly lower left ventricular end-diastolic dimension (3.83 vs 4.5, P = 0.008), left ventricular end-systolic dimension (2.33 vs 2.97, P = 0.017), right ventricular fraction area change (38.6 vs 48.7, P = 0.025), and left ventricular outflow tract (1.66 vs 2.02, P = 0.020) and higher systolic pulmonary arterial pressure (57 vs 32.4, P = 0.008). At 1 and 6 months, there were no significant differences in echocardiographic parameters between the significant and insignificant AR groups' left ventricular end-diastolic dimension (4.58 vs 4.5, 4.64 vs 4.57), left ventricular end-systolic dimension (3.1 vs 2.9, 2.9 vs 2.91), and systolic pulmonary arterial pressure (28 vs 34.7, 33.8 vs 32.4). Those with significant AR had a higher mean Wilkin score (mean, 10; P = 0.007) and subvalvular thickening (mean, 2.6; P = 0.005).

CONCLUSION

On short-term follow-up, the presence of mild to moderate AR before PTMC does not lead to severe AR. The outcomes regardless of the group showed a good functional class and no significant echocardiographic differences when compared.

Rheumatic Heart Disease ; Mitral Valve Stenosis

Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue that results from abnormal fibrillin-1. It is caused by a mutation in the FBN1 gene making it a multisystemic disease. It is the most common but may have overlap with other thoracic aortic syndromes. It is primarily diagnosed using the 2010 revised Ghent nosology criteria that rely on the presence of family history, progressive aortic root dilatation or dissection, ectopia lentis, genetic testing, and systemic features. Unfortunately, a lack of nationwide awareness, family history, and medical access may lead to delayed diagnosis and treatment in our setting.

Case Summary: We report 4 cases of Marfan Syndrome from May to October 2019, which were seen at our institution. They presented with either recently diagnosed aortic aneurysm, dissection, or severe heart failure. We used the Revised Ghent Nosology for diagnosis and treated them with the standard of care. However, outcomes were different across patients.

Discussion: The life expectancy is comparable to the general population If early diagnosis is achieved. A myriad of known cardiovascular complications can manifest from severe valvular heart disease secondary to mitral valve prolapse, aortic root dilatation, and, most commonly, aortic dissection.

Conclusion: A late diagnosis presenting as aortic dissection or severe valvular heart disease may have a poor quality of life and prognosis. A thoracic aortic syndrome, most commonly Marfan, should still be considered in patients with aortic criterion and premature aortic syndrome even if a systemic score of 7 is not fulfilled. The screening of first-degree relatives of affected patients is an effective strategy to diagnose them at an early stage where they are still asymptomatic.

Marfan Case Series ; Premature Aortic Disease ; Thoracic Aortic Syndromes