Hypertrophic obstructive cardiomyopathy complicated by ruptured chordae tendinea to the posterior mitral leaflet and severe congestive heart failure

Abstract

H ypertrophic obstructive cardiomyopathy (HOCM) is frequently associated with mitral valve regurgitation (MR). In most instances, the latter is related to functional left ventricular outflow tract (LVOT) obstruction rather than intrinsic disorders of the mitral valve. The latter, however, need to be considered in presentations that are inconsistent with the rather stable natural history of HOCM. Mitral valve prolapse (MVP), with or without chordal rupture, can complicate HOCM by adding to the burden of MR and thereby aggravating and/or precipitating congestive heart failure. CASE PrESEntAtionA 63-year-old woman presented to the emergency department because of progressive, painless shortness of breath. She had suffered from fatigue for the past two weeks, shortness of breath on a home treadmill for three days, and dyspnea at rest for one day. Previous dyspnea, chest discomfort, ischemic heart disease, palpitations, presyncope or syncope were denied. She was hypertensive and had had 'asthma' for approximately 10 years. Three years before admission, a transthoracic echocardiogram revealed symmetrical left ventricular hypertrophy with hyperdynamic left ventricular systolic function and systolic anterior motion (SAM) of the mitral valve. Eccentric MR of uncertain severity was presumed to be secondary to HOCM.The patient was taking 2.5 mg of ramipril, 25 mg of hydrochlorothiazide and 240 mg of diltiazem daily.She was short of breath at rest. Her blood pressure was 162/79 mmHg and her pulse was 123 beats/min and regular. Her respiratory rate was 30 breaths/min. A grade 4/6 systolic crescendo-decrescendo murmur was noted. The jugular venous pressure was normal. The chest was clear. There was no edema.The electrocardiogram revealed sinus tachycardia at 114 beats/min, borderline left atrial abnormality and voltage criteria for left ventricular hypertrophy. The chest x-ray revealed congestive heart failure with bilateral pleural effusions. The laboratory data were unremarkable.The patient was admitted and treated with oxygen and diuretics. Her dyspnea and tachycardia persisted. Low-dose metoprolol was added but shortness of breath continued. Her clinical response was believed to be inconsistent with hypertrophic cardiomyopathy (HCM) alone. An urgent transthoracic echocardiogram revealed MR with probable prolapse of the posterior leaflet of the mitral valve. There was marked SAM of the mitral valve with a peak instantaneous LVOT gradient of 148 mmHg. A transesophageal echocardiogram examination revealed asymmetrical septal hypertrophy (septal thickness 17 mm), SAM of the mitral valve, ruptured chordae to the posterior leaflet of the mitral valve and severe MR. There was no evidence of bacterial endocarditis.The coronary angiogram was normal. Surgery revealed ruptured chordae to the P2 segment of the posterior leaflet, with mild thickening of the anterior leaflet. The mitral valve was replaced using a St Jude's prosthesis (#29; St Jude Medical Inc, USA). A septal myomectomy was not performed. The intraoperative LVOT gradient...

Subaortic Membrane With Mitral Chordae Rupture Mimicking Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

“…These resulted in acute MR, and amelioration of the LVOT obstruction. This contrasts with the findings in our case, where chordae rupture was found with consistent SAM and LVOT gradient [3]. Four-dimensional CT can be used in such cases for the assessment of myocardial hypertrophy and the evaluation of exact mitral subvalvular abnormalities [4].…”

Complete Resolution of Left Ventricular Outflow Tract Obstruction After Spontaneous Mitral Valve Chordal Rupture in a Patient With Hypertrophic Cardiomyopathy

“… 2 , 3 A literature search found 14 cases of HOCM with ruptured chordae tendineae ( Table 1 ). 2 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 In the present case, ruptured chordae tendineae were confirmed by transesophageal echocardiography. We were not able to identify any specific causes of chordal rupture, such as myocardial infarction, infective endocarditis, or trauma.…”

“…Chordal rupture in HOCM has been reported to cause two major hemodynamic changes. One is the negative effect of acute MR, and the other is the positive effect of alleviation of LVOT obstruction 4, 7, 8, 9, 10, 11. Most of the previously reported cases had acute deterioration of HF due to acute MR, requiring emergent or urgent surgical treatment.…”

“…One is the negative effect of acute MR, and the other is the positive effect of alleviation of LVOT obstruction. 4 , 7 , 8 , 9 , 10 , 11 Most of the previously reported cases had acute deterioration of HF due to acute MR, requiring emergent or urgent surgical treatment. However, one case was reported to be clinically stable, and left ventricular wall thickness decreased after chordal rupture.…”

Clinical case of acute chord rupture of the mitral valve posterior leaflet in older Patient with co-morbidities

“…Since that time, there have been described many cases of chords ruptures and related to it severe mitral regurgitation and progression of heart failure [9, 573; 10, 253]. Chordal rupture complicate chronic rheumatic valvular disease (mostly during the chronic stage) [1, 134; 6, 121], bacte rial endocarditis on both normal and rheumatic valves [2, 297; 6, 121], degenerative mitral valve disease, ischemic heart disease, acute myocardi al infarction, non-mitral heart and valvular ab normalities (such as aortic stenosis and aortic regurgitation), it m ay be in patients with systolic hypertension [5, 978; 8, 77], systemic or gener alized connective tissue abnormality (Marfan's and the Ehlers-Danlos syndromes, osteogenesis imperfect), after blunt chest trauma or surgery [12,257], in patient with systemic lupus erythe matosus, congenital heart disease, hypertrophic obstructive cardiomyopathy [11,259], systol ic click-late systolic murmur (mitral valve pro lapse) syndrome and in association with preg nancy. Acute mitral regurgitation resulting from rupture of chordae tendineae has also been re ported in those who engage in vigorous exercises [3,256] (Table 1).…”