Prevalence and characteristics of disproportionate ventricular septal thickening in patients with acquired or congenital heart diseases: echocardiographic and morphologic findings.

Maron, Barry J.; Clark, Chester E.; Henry, Walter L.; Fukuda, Toyoki; Edwards, Jesse E.; Mathews, Emmett C.; Redwood, David R.; Epstein, Stephen E.

doi:10.1161/01.cir.55.3.489

Cited by 91 publications

(28 citation statements)

References 31 publications

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“…In most cases, the hypertrophy is concentric, although some patients present with an asymmetric upper septal hypertrophy with or without dynamic LVOT obstruction. [1][2][3] Our patient demonstrated severe asymmetric upper septal hypertrophy, but had no evidence of LVOT obstruction on dynamic preoperative testing. Upon implantation of the transcatheter prosthesis, however, she developed significant LVOT obstruction.…”

Section: Discussionmentioning

confidence: 62%

Combined Transcatheter Aortic Valve Replacement and Emergent Alcohol Septal Ablation

et al. 2013

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A 91-year-old woman presented with worsening exertional dyspnea. Her previous medical history included hypertension, percutaneous coronary intervention with drugeluting stent placement to the mid left anterior descending coronary artery, moderate bilateral carotid atherosclerosis, and moderate chronic obstructive pulmonary disease.Transthoracic echocardiography revealed a normal left ventricular ejection fraction with asymmetric upper septal hypertrophy (posterior wall, 1.3 cm; interventricular septum, 2.0 cm; Figure 1A) but without left ventricular outflow tract (LVOT) obstruction ( Figure 1B) at rest or with a Valsalva maneuver. There was severe aortic valve calcification and stenosis ( Figure 1A and 1C).The patient was referred for transcatheter aortic valve replacement (AVR) via the transfemoral approach. Upon crossing the stenotic aortic valve, the peak-to-peak left ventricle-aorta (LVAo) pressure gradient was recorded at 60 mm Hg (Figure 2A). After deployment of a 23-mm Edwards SAPIEN prosthesis, the patient became hypotensive and the peak-to-peak LV-Ao gradient was recorded at 120 mm Hg ( Figure 2B). Transesophageal echocardiography demonstrated a properly functioning aortic prosthesis and revealed a dynamic obstruction in the LVOT attributable to systolic anterior motion of the mitral valve ( Figure 3A), associated with severe mitral regurgitation ( Figure 3B).After review of the preprocedural coronary angiogram ( Figure 4A), the decision was made to proceed emergently to alcohol septal ablation to relieve the dynamic LVOT obstruction. Isopropyl alcohol (1.5 mL) was infused in the septal perforator, and subsequent angiography revealed occlusion of the vessel ( Figure 4B). Transesophageal echocardiography revealed marked reduction in systolic anterior motion and in the degree of LVOT obstruction and mitral regurgitation, as well ( Figure 5). Hemodynamic tracings demonstrated almostcomplete resolution of the LV-Ao gradient ( Figure 6).The patient was successfully extubated at the conclusion of the case and was discharged from the hospital 5 days later. At 1-month follow-up, transthoracic echocardiography revealed continued reduction in the LV-Ao gradient at rest (15 mm Hg) and minimal increase with provocation by a Valsalva maneuver (18 mm Hg). DiscussionIn patients with aortic stenosis, hypertrophy of the left ventricular myocardium often occurs as a result of the increased myocardial load imposed by the valvular obstruction. In most cases, the hypertrophy is concentric, although some patients present with an asymmetric upper septal hypertrophy with or without dynamic LVOT obstruction. 1-3Our patient demonstrated severe asymmetric upper septal hypertrophy, but had no evidence of LVOT obstruction on dynamic preoperative testing. Upon implantation of the transcatheter prosthesis, however, she developed significant LVOT obstruction. This is likely the result of a number of physiological and anatomic changes. The relationship between afterload and LVOT obstruction has been elucidated by numerous investigators...

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Section: Discussionmentioning

confidence: 62%

Combined Transcatheter Aortic Valve Replacement and Emergent Alcohol Septal Ablation

et al. 2013

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“…13 The definition of septal hypertrophy in Noonan syndrome is complicated by the frequent association of valvular pulmonary stenosis, which may result in asymmetrical septal hypertrophy and thus mimic HCM. 14 In this case, the marked hypertrophy was thought to be the primary manifestation of HCM, but not the secondary hypertrophy, because both the ventricular free walls, as well as the interventricular septum, were distinctly hypertrophied without evidence of valvular stenosis. Moreover, the histopathology of the endomyocardial biopsy specimens was consistent with HCM.…”

Section: Discussionmentioning

confidence: 76%

Biventricular Hypertrophic Cardiomyopathy With Right Ventricular Outflow Tract Obstruction Associated With Noonan Syndrome in an Adult

et al. 2001

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oonan syndrome is one of the most common nonchromosomal syndromes frequently accompanied by cardiovascular malformations. 1 The major cardiovascular defects are valvular pulmonary stenosis, commonly due to a dysplastic valve, 2,3 and hypertrophic cardiomyopathy (HCM), which predominantly affects the interventricular septum and left ventricle. [4][5][6][7][8][9] We report an adult case of Noonan syndrome with biventricular HCM accompanied by isolated right ventricular outflow tract (RVOT) obstruction without valvular abnormalities. To the best of our knowledge, these conditions are regarded as rather uncommon manifestations of cardiac defects in adults with Noonan syndrome. Case ReportA 42-year-old man with dyspnea and palpitation on exertion was referred from his local hospital for further cardiac examination in April 2000. Heart murmur and electrocardiographic abnormalities were first noticed when he was 10 years of age, and he had experienced mild dyspnea on exertion since that time. On admission, blood pressure was 118/68 mmHg and pulse was regular at 72 beats/min. Neither mental retardation nor sexual infantilism was observed. He was of short stature (152cm; < mean -3.0 SD), had a short neck, a shield-like chest with widely spaced nipples, cubitus valgus (Fig 1A), and the characteristic facial traits of ocular hypertelorism and low-set posteriorly rotated ears ( Fig 1B). A grade 3 systolic ejection murmur was heard at the mid-left sternal border and S4 was heard at the apex. An electrocardiogram showed left axis deviation, P waves with high peaks in the inferior limb leads, and deep S waves over the precordial leads. Chest X-ray showed cardiomegaly with normal pulmonary vascularity. Laboratory tests revealed prolongation of the activated partial thromboplastin time (46.2 s; normal value, 24-37 s) and a somewhat deficient clotting factor 11 (61.2%; normal value, 75-137%) but without any hepatic dysfunction. The lymphocyte karyotype showed a normal male pattern.Transthoracic echocardiography (Fig 2) revealed biventricular hypertrophy with asymmetric septal hypertrophy. The hypertrophied septum bulged out into the outflow portion of the right ventricle, thus reducing the size of the right ventricular cavity and resulting in outflow obstruction. Transesophageal echocardiography (Fig 3) demonstrated far more pronounced hypertrophy in the interventricular septum than in the free wall of each ventricle and marked narrowing of the RVOT. The pulmonary valve was normal. Magnetic resonance imaging clearly demonstrated marked biventricular hypertrophy including that of the left ventricular free wall (Fig 4). Cardiac catheterization showed a pulmonary capillary wedge pressure of 11 mmHg, pulmonary arterial pressure of 22/12 (16) mmHg, RVOT pressure of 22/6 mmHg, right ventricle apex pressure of 70/6 mmHg, right atrial pressure of 6 mmHg, systemic arterial pressure of 110/70 (90) mmHg, and cardiac index of 1.44 L·min -1 · m -2 . A systolic pressure gradient of 48 mmHg was seen across the right ventricular outflow tract (F...

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“…However, Fulton's method makes no account of pulmonary hypertension-related asymmetric septal hypertrophy (21,38,39), which becomes more common and severe as pulmonary pressure rises (39) and regresses with treatment (pre-single-lung transplant IVS ¼ 1.3 cm, post-¼ 0.9 cm (3)). This phenomenon has been attributed to hypertrophy of ''RV'' septal components (40).…”

Section: Discussionmentioning

confidence: 99%

Measuring the heart in pulmonary arterial hypertension (PAH): Implications for trial study size

Bradlow

Hughes

Keenan

et al. 2009

Magnetic Resonance Imaging

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Purpose: To calculate the sample size for a theoretical pulmonary arterial hypertension (PAH) randomized controlled trial (RCT) by using cardiovascular magnetic resonance (CMR) imaging to determine the repeatability of measures between two scans. Materials and Methods:Two same-day examinations from 10 PAH patients were analyzed manually and semiautomatically. Study size was calculated from the standard deviation (SD) of repeatability. Different approaches to right-ventricle (RV) mass were investigated, agreement between methods tested and interobserver reproducibility measured by Bland-Altman analysis to explore how the PAH heart might be best measured.Results: Repeatability was good for almost all manuallymeasured indices but poor for semiautomated measurement of RV mass and left-ventricle (LV) end-diastolic volume (EDV). Thus, for an RCT (power, 80%; significance level, 5%) analyzing ''outcome'' indices (RVEDV, LVEDV, RV ejection fraction, and RV mass; anticipated change: 10 mL, 10 mL, 3%, and 10 g, respectively) manually, 34 patients are required compared to 78 if analysis is semiautomated. RV mass was repeatable if the interventricular septum was divided between ventricles or if wholly apportioned to the LV. Limits of agreement between manual and semiautomated analyses were unsatisfactory for RV measures and interobserver reproducibility was worse for semiautomated than manual analysis. Conclusion:Manual is more robust than semiautomated analysis and at present should be favored in RCTs in PAH as it leads to lower sample size requirements.

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Prevalence and characteristics of disproportionate ventricular septal thickening in patients with acquired or congenital heart diseases: echocardiographic and morphologic findings.

Cited by 91 publications

References 31 publications

Combined Transcatheter Aortic Valve Replacement and Emergent Alcohol Septal Ablation

Combined Transcatheter Aortic Valve Replacement and Emergent Alcohol Septal Ablation

Biventricular Hypertrophic Cardiomyopathy With Right Ventricular Outflow Tract Obstruction Associated With Noonan Syndrome in an Adult

Measuring the heart in pulmonary arterial hypertension (PAH): Implications for trial study size

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