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Childhood-onset cardiomyopathies represent a rare and poorly understood subset of cardiac disorders. In a recent study published in the European Heart Journal, investigators delved into the demographics and prognosis of pediatric cardiomyopathy patients within the inaugural European Cardiomyopathy Registry. months (IQR: 11.3–15.3),
Idiopathic dilated cardiomyopathy Idiopathic dilated cardiomyopathy is a form of dilated cardiomyopathy with unknown etiology characterised by progressive left ventricular dilatation and systolic dysfunction. The incidence of idiopathic dilated cardiomyopathy varies from 3-10 cases per 100,000 population.
Hypertrophic cardiomyopathy is a genetic disorder with a guarded prognosis which occurs in about 1:500 individuals. The most common symptom of hypertrophic cardiomyopathy is dyspnoea which occurs in 90% of cases and is due to elevated left ventricular diastolic pressures as a consequence of the diastolic dysfunction.
2024 ACC/AHA/Multisociety Guidelines for Management of Hypertrophic Cardiomyopathy is now available online with free access at JACC website. Those who are in a hurry could go through the 2024 Hypertrophic Cardiomyopathy Guideline-at-a-Glance which is a five page document which summarises top ten take home messages from the guidelines.
The primary outcome is the GDMT prescription rate score, with secondary outcomes covering the time to full GDMT optimization, patient and clinician satisfaction, time spent on healthcare, and the Kansas City Cardiomyopathy Questionnaire.
Mavacamten is now approved for treatment of symptomatic hypertrophic obstructive cardiomyopathy and endorsed by mult-society guidelines. Phase 2 Study of Aficamten in Patients With Obstructive Hypertrophic Cardiomyopathy was published in 2023, with 28 patients on aficamten 13 on placebo. J Am Coll Cardiol. 2023 Jan 3;81(1):34-45.
When non-compaction is associated with left ventricular dysfunction it is called left ventricular non-compaction cardiomyopathy. Blood flow into and out of these recesses can provide a spectacular view on colour Doppler echocardiography, almost looking like flames of fire near the apex, in the apical four chamber view.
Enlargement of the left ventricular cavity, increased wall thickness and increased trabeculations in athlete’s heart will have to be differentiated from conditions like dilated cardiomyopathy, hypertrophic cardiomyopathy and isolated left ventricular non-compaction. Differentiating Athlete’s Heart From Cardiomyopathies – The Left Side.
It is mentioned that square root sign can also occur in right right ventricular cardiomyopathy and severe bradycardia [1]. Reference Doshi S, Ramakrishnan S, Gupta SK. Invasive hemodynamics of constrictive pericarditis. Indian Heart J. 2015 Mar-Apr;67(2):175-82. doi: 10.1016/j.ihj.2015.04.011. 2015.04.011.
Here it is 1 and in cases like asymmetric septal hypertrophy or hypertrophic cardiomyopathy, there will be disproportionate thickening of the septum. IVSd is thickness of interventricular septum in diastole and EDV end diastolic volume. IVS/LVPW is the ratio between thickness of these two. ESV is end systolic volume.
A triphasic left ventricular filling pattern with an additional mid diastolic wave, called T wave by some authors and L wave by others, can occur in situations of left ventricular diastolic dysfunction, especially in hypertrophic cardiomyopathy. Another video on this channel describes triphasic mitral flow in more detail.
Patients receiving semaglutide showed a greater change in Kansas City Cardiomyopathy Questionnaire (KCCQ) clinical summary scores at 52 weeks than placebo. Investigators randomized 616 adult patients with HFpEF and obesity to receive either once weekly semaglutide (n=310) or placebo (n=306).
Though echocardiography is the sheet anchor of diagnosis of hypertrophic cardiomyopathy, clinical evaluation is equally important while deciding management and ruling out differential diagnoses. Role of cardiac cath is now mostly limited to septal ablation.
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