The Natural History of Mild Congenital Aortic Stenosis in Patients Studied by Serial Left Heart Catheterization.

Introduction: Congenital aortic stenosis (AS) has been reported to manifest a slow rate of progression in mild disease with a greater likelihood of progression in patients with moderate-severe disease. The natural history of the Doppler estimated peak gradient (DEPG) in patients after balloon aortic valvuloplasty (BAV) has not previously been studied on a large scale. Methods: A retrospective review was performed of 360 patients from 1984-2012 with AS providing a total of 2051 echocardiograms before and after BAV. Patients were excluded if they had an intervention within the first 30 days of life. The relationships between the AS DEPG and several predictors (age at time of initial echocardiogram, valve morphology, and history of intervention) were explored using linear mixed effect models. The DEPG slope was then calculated in patients who had at least 2 echocardiograms before and after balloon dilation using linear regression modeling. Results: The rate of increase in the DEPG for all patients with AS was 5.6 mmHg per 10 years of age (p<0.001). The DEPG increased over time regardless of age at presentation with the greatest mean increase in patients presenting from 10-14.9 years (n=59; 11.9 mmHg per 10 years; p<0.001). Patients who went on to have a BAV or surgical intervention on the aortic valve had a significantly higher rate of AS progression than the overall patient cohort (n=59; 18.0 mmHg/10 years and n=36; 13.1 mmHg/10 years). Patients with a unicommissural (n=39) aortic valve had a significantly higher rate of progression compared to those with a bicommissural (n=270) aortic valve (8.1 mmHg/10 years and 4.5 mm Hg/10 years; p<0.001). The median rate of progression in the post-BAV group was significantly lower than the median pre-BAV rate of progression (n=34; pre-BAV 3.97 (1.69-8.7) mmHg/year; post-BAV 0.40 (-1.80-3.88) mmHg/year; p<0.01). Conclusions: The DEPG of native valve congenital aortic stenosis shows a slow, linear rate of progression prior to intervention. The rate of progression is significantly higher in patients with a unicommissural aortic valve as well as those patients that go on to have a BAV and/or surgical intervention. The rate of the DEPG progression is significantly lower after BAV.

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New technique for retrograde left heart catheterization in aortic stenosis

Catheterization and Cardiovascular Diagnosis ◽

10.1002/ccd.1810080315 ◽

1982 ◽

Vol 8 (3) ◽

pp. 299-304 ◽

Cited By ~ 10

Author(s):

Hans R. Baur ◽

Gerald L. Mruz ◽

David L. Erickson ◽

Robert L. Vantassel

Keyword(s):

Aortic Stenosis ◽

New Technique ◽

Heart Catheterization ◽

Left Heart ◽

Left Heart Catheterization

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Multi-vessel coronary artery aneurysms in a patient with Parry Romberg syndrome: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz103 ◽

2019 ◽

Vol 3 (3) ◽

Author(s):

Andrew Mehlman ◽

Jaymin Patel ◽

Christopher Bitetzakis ◽

Michael Berlowitz

Keyword(s):

Coronary Artery ◽

Underlying Mechanism ◽

Heart Catheterization ◽

Left Heart ◽

Coronary Artery Aneurysms ◽

Left Heart Catheterization ◽

History Of ◽

Parry Romberg Syndrome ◽

Vessel Coronary Artery

Abstract Background Coronary artery aneurysms (CAAs) are a very rare finding on coronary angiograms with multiple known aetiologies. Parry Romberg syndrome (PRS) is also a very rare disease, and the underlying aetiology remains unknown. We present a rare case of CAAs in a patient with PRS, and discuss possible implications regarding the primary pathophysiological cause for both of these diseases. Case summary A 48-year-old woman with a history of PRS presented with atypical and non-exertional chest pain. Initial evaluation demonstrated a rising troponin without associated electrocardiogram changes, and as such she was taken for left heart catheterization. Left heart catheterization demonstrated diffuse aneurysmal and ectatic disease of multiple coronary arteries. Further evaluation with magnetic resonance angiogram and autoantibody panel did not demonstrate other vascular anomalies or rheumatologic disease, respectively. She was treated with dual anti-platelet therapy and statin, and at 1 year follow-up, she had resolution of her symptoms. Discussion It has been postulated that the underlying mechanism causing CAA is intravascular inflammation. Parry Romberg syndrome is theorized to be a neurovasculopathy, as evidenced by cases of associated intracranial aneurysms. Intravascular inflammation may play a key pathological role in CAA, and an association between CAA and PRS may exist.

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Constrictive pericarditis in the setting of repeated chest trauma in a mixed martial arts fighter

BMC Cardiovascular Disorders ◽

10.1186/s12872-021-02378-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Meganne N. Ferrel ◽

Sentia Iriana ◽

I. Raymond Thomason ◽

Christy L. Ma ◽

Katsiaryna Tsarova ◽

...

Keyword(s):

Constrictive Pericarditis ◽

Martial Arts ◽

Venous Pressure ◽

Fibrous Tissue ◽

Heart Catheterization ◽

Delayed Presentation ◽

Left Heart ◽

Mixed Martial Arts ◽

Left Heart Catheterization ◽

History Of

Abstract Background Constrictive pericarditis (CP) is characterized by scarring and loss of elasticity of the pericardium. This case demonstrates that mixed martial arts (MMA) is a previously unrecognized risk factor for CP, diagnosis of which is supported by cardiac imaging, right and left heart catheterization, and histological findings of dense fibrous tissue without chronic inflammation. Case presentation A 47-year-old Caucasian male former mixed martial arts (MMA) fighter from the Western United States presented to liver clinic for elevated liver injury tests (LIT) and a 35-pound weight loss with associated diarrhea, lower extremity edema, dyspnea on exertion, and worsening fatigue over a period of 6 months. Past medical history includes concussion, right bundle branch block, migraine headache, hypertension, chronic pain related to musculoskeletal injuries and fractures secondary to MMA competition. Involvement in MMA was extensive with an 8-year history of professional MMA competition and 13-year history of MMA fighting with recurrent trauma to the chest wall. The patient also reported a 20-year history of performance enhancing drugs including testosterone. Physical exam was notable for elevated jugular venous pressure, hepatomegaly, and trace peripheral edema. An extensive workup was performed including laboratory studies, abdominal computerized tomography, liver biopsy, echocardiogram, and cardiac magnetic resonance imaging. Finally, right and left heart catheterization—the gold standard—confirmed discordance of the right ventricle-left ventricle, consistent with constrictive physiology. Pericardiectomy was performed with histologic evidence of chronic pericarditis. The patient’s hospital course was uncomplicated and he returned to NYHA functional class I. Conclusions CP can be a sequela of recurrent pericarditis or hemorrhagic effusions and may have a delayed presentation. In cases of recurrent trauma, CP may be managed with pericardiectomy with apparent good outcome. Further studies are warranted to analyze the occurrence of CP in MMA so as to better define the risk in such adults.

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