Brynn Connor

All Publications

• Prevalence and Outcomes of Primary Left Ventricular Dysfunction in Marfan Syndrome. The American journal of cardiology Connor, B. S., Algaze, C. A., Narkeviciute, A., Anguiano, B., Pariani, M., Zarate, Y. A., Collins, R. T. 2022

  Abstract

  Even in the absence of significant valvular disease, patients with Marfan syndrome (MFS) have evidence of impaired left ventricular (LV) performance, suggestive of a primary cardiomyopathy. However, the true prevalence and long-term outcomes of this disease process remain largely unknown. We performed a retrospective analysis of all adult patients with confirmed MFS followed at Stanford Health Care. Those with significant valvular regurgitation, coronary artery disease, or previous cardiac surgery were excluded. LV systolic dysfunction was defined as a LV ejection fraction (LVEF) <55% on transthoracic echocardiography. A total of 753 patients with confirmed MFS were followed up over a median duration of 8 years (interquartile range 4 to 13). Of those, 241 patients (53% women, 71% White) met inclusion criteria and comprised the study cohort. LV systolic dysfunction was present in 30 patients (12%), with a median age of onset of 25 years (interquartile range 19 to 37), median EF of 52% (interquartile range 48 to 54), and evidence of clinical heart failure (New York Heart Association functional class ≥II) in 10% of patients. LV systolic dysfunction was more common in patients with larger aortic root diameters (≥4.0 cm: Odds ratio=4.5, 95% confidence interval=1.2 to 17.1) but was not associated with other cardiovascular manifestations of MFS or traditional atherosclerotic risk factors. In conclusion, apart from significant valvular pathology, LV systolic dysfunction was prevalent in MFS from a young age, suggestive of a primary cardiomyopathy. LV dysfunction was typically mild and subclinical and occurred more commonly in patients with more pronounced aortopathies.

  View details for DOI 10.1016/j.amjcard.2022.04.012

  View details for PubMedID 35550817

• A clinical scoring system for early onset (neonatal) Marfan syndrome. Genetics in medicine : official journal of the American College of Medical Genetics Zarate, Y. A., Morris, S. A., Blackshare, A., Algaze, C. A., Connor, B. S., Kim, A. J., Yutzey, K. E., Miller, E. M., Weaver, K. N., Collins, R. T. 2022

  Abstract

  PURPOSE: This study aimed to develop objective diagnostic criteria for early onset Marfan syndrome (eoMFS) to facilitate early diagnosis and timely interventions.METHODS: On the basis of an extensive literature review and the responses from a survey distributed among providers with expertise in the diagnosis and management of eoMFS, we developed an age-based, diagnostic scoring system encompassing 10 features common to eoMFS (9 clinical+ 1 laboratory) and divided them into cardiac, systemic, and FBN1 (on the basis of the location of the pathogenic FBN1 variant) scores.RESULTS: In total, 77 individuals with eoMFS (13 newly reported) and 49 individuals diagnosed with classical Marfan syndrome during early childhood were used to validate the criteria. Median cardiac (8 vs 0, P < .001), systemic (11 vs 3, P < .001), FBN1 (5 vs 0, P < .001), and total (23 vs 4, P < .001) scores were significantly higher in individuals with eoMFS than in those without. A proposed clinical score (cardiac+ systemic) cutoff of ≥14 points showed excellent sensitivity (100%), specificity (92%), and reliability (correctly classified= 94%).CONCLUSION: Distinct from classical Marfan syndrome in phenotype and morbidity, eoMFS can be diagnosed clinically using an objective scoring system encompassing the typical physical features and cardiac disease manifestations. Although genetic testing can be suggestive of eoMFS, genetic testing alone is insufficient for diagnosis.

  View details for DOI 10.1016/j.gim.2022.03.016

  View details for PubMedID 35420547

• A clinical scoring system for early onset (neonatal) Marfan syndrome Zarate, Y., Morris, S., Blackshare, A., Algaze, C., Connor, B., Kim, A., Yutzey, K., Miller, E., Weaver, K., Collins, T. ELSEVIER SCIENCE INC. 2022: S353

  View details for DOI 10.1016/j.gim.2022.01.573

  View details for Web of Science ID 000796586200313

• Factors Associated With Increased Exercise in Adults With Congenital Heart Disease. The American journal of cardiology Connor, B., Osborne, W., Peir, G., Smith, M., John, A. 2019

  Abstract

  Despite the known benefits of regular exercise, the majority of adults with congenital heart disease (CHD) fail to meet recommended standards for physical activity. We aimed to evaluate the factors associated with exercise frequency in adults with CHD, which remain largely unknown. From September 2015 to December 2016, 446 adults with CHD completed clinical questionnaires regarding exercise frequency. Questionnaires also measured related demographic and psychosocial variables. Retrospective chart review was utilized to determine cardiac function and cardiopulmonary exercise capacity. Exercise frequency was classified as none (33%), low (<3*/month, 2%), occasional (<2*/week, 8%), or frequent (≥2*/week, 57%). Frequent exercisers were more highly educated (odds ratio [OR] 1.65, 95% confidence interval [CI] 1.06 to 2.57), reported improved sleep quality, with decreased nocturnal awakenings and sleep latency (OR 0.52, 95% CI 0.32 to 0.83), and had a lower prevalence of depression (OR 0.30, 95% CI 0.12 to 0.75). There was no significant association between exercise frequency and disease complexity or cardiac function. Of the original cohort, 74 patients completed exercise testing within 1 year of their visit. Frequent exercisers (n = 46) had better indicators of cardiovascular capacity, including higher peak maximal oxygen consumption [VO2] (28.8 ± 8.5 vs 24.6 ± 8.4, p = 0.04). After controlling for exercise frequency, higher educational attainment independently predicted an improved exercise capacity (peak [VO2]: OR = 1.09, 95% CI 1.01 to 1.18). In conclusion, frequent exercise in adults with CHD is associated with improved cardiopulmonary exercise capacity and psychosocial functioning, irrespective of underlying cardiac disease complexity and severity. Increased physical activity levels and improved exercise capacity were observed in more highly educated patients, highlighting the potential importance of socioeconomic influences on physical and mental functioning.

  View details for DOI 10.1016/j.amjcard.2019.05.073

  View details for PubMedID 31327487

• Ovulation Suppression to Prevent Hemoperitoneum and Surgical Menopause in Anticoagulated Women. JACC. Case reports Connor, B. S., Coviello, E., Fries, M., John, A. S. 2019; 1 (1): 50-54

  Abstract

  Premenopausal women taking anticoagulation therapy are at risk of developing hemorrhagic ovarian cysts. This paper presents 3 cases of acute hemoperitoneum, with resultant surgical menopause, secondary to cystic hemorrhage in premenopausal women with repaired congenital heart disease (CHD). Adults with CHD taking long-term anticoagulation should be considered candidates for ovulation suppression with hormone-based contraception. (Level of Difficulty: Intermediate.).

  View details for DOI 10.1016/j.jaccas.2019.05.014

  View details for PubMedID 34316741

  View details for PubMedCentralID PMC8288976