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The Non-Ischemic HEART FAILURE Patient
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Maria-Anna Bazmpani, Theodoros D. Karamitsos
Restrictive cardiomyopathy is a rare type of cardiomyopathy. It is defined as restrictive ventricular physiology in the presence of normal or reduced diastolic volumes, normal or reduced systolic volumes, and normal ventricular wall thickness.8 As disease progresses, patients present with systemic and pulmonary venous congestion, and atrial fibrillation, and have a poor prognosis.24 See also Table 13.2.
Cardiomyopathies in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Restrictive cardiomyopathies are characterized by the presence of “restrictive filling pattern in the presence of normal or reduced diastolic volumes, normal or reduced systolic volumes, and normal wall thickness” and may represent various pathologies rather than a distinct entity [2]. Primary restrictive cardiomyopathy may be inherited due to genetic mutations in cardiac proteins, such as troponin, or non-inherited, such as in infiltrative disorders including hemochromatosis, amyloidosis, or sarcoid or radiation exposure [2]. There are only rare case reports of pregnancy outcomes with restrictive cardiomyopathy [76–78]. Some recommend that pregnancy be avoided in symptomatic patients [28].
Cardiac and cardiovascular disorders
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
Restrictive cardiomyopathy is often neither genetic nor familial but can be caused by amyloidosis, endomyocardial fibrosis and a number of genetic and non-genetic conditions. The information relevant for genetic counselling will depend upon the underlying diagnosis (when this can be determined), by the geographical region and by the population of origin of the affected individual.
Advances in multi-modality imaging for constrictive pericarditis and pericardial inflammation: role of imaging-guided therapy
Published in Expert Review of Cardiovascular Therapy, 2023
Tahir S Kafil, Tom Kai Ming Wang, Ankit Agrawal, Muhammad Majid, Alveena B Syed, Erika Hutt, Ben Alencherry, Joshua A Cohen, Sachin Kumar, Agam Bansal, Brian P Griffin, Allan L Klein
CMR strain is being evaluated in the research setting. A recent study by Yang et al. assessed 32 patients with CP by CMR-feature tracking strain imaging [31]. They compared CP to restrictive cardiomyopathy and controls. They found distinct diastolic patterns with average time-strain curves in CP. CP had a plateau pattern with rapid down and plateau of the time-strain curve from start of diastole to passive filling, thereafter, slowing toward baseline at end diastole. This was distinct from restrictive cardiomyopathy that showed slow and steady gradual decline toward baseline during all of diastole. Normal controls had rapid decrease at start of diastole with a fast downwards at end diastole. This showed that novel CMR-feature tracking could differentiate between CP and restrictive cardiomyopathy based on time-strain curves.
AL type cardiac amyloidosis: a devastating fatal disease
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
Adeel Nasrullah, Anam Javed, Thejus T Jayakrishnan, Aaron Brumbaugh, Ariel Sandhu, Brent Hardman
Clinical presentation of AL type cardiac amyloidosis is varied based on the involved site. Fatigue and weakness are the most common presenting symptoms. Restrictive cardiomyopathy presents with signs and symptoms of diastolic heart failure and decreased exercise tolerance. With progression of the disease, atrial dilation occurs, which predisposes patients to atrial fibrillation and further sequelae of clot formation and systemic embolization. Cardiac conduction may be disrupted by amyloid deposition, often causing a variety of heart blocks. Soft tissue involvement has been seen as periorbital ecchymosis and macroglossia in 12.5% and 27.2%, respectively [7]. Renal AL amyloid can cause myeloma kidney and nephrotic syndrome. With underlying kidney disease, the patient may develop progressive renal failure requiring renal replacement therapy, as seen in the present case. Similar deposits in the liver and peripheral nerves can present as hepatomegaly, transaminitis, and peripheral neuropathy, respectively.
A review of the criteria for non-invasive diagnosis of cardiac transthyretin amyloidosis
Published in Expert Opinion on Orphan Drugs, 2021
Tamer Rezk, Marianna Fontana, Julian D. Gillmore
Systemic amyloidosis is a rare disease caused by the extracellular deposition of amyloid, a fibrillar material, derived from a variety of precursor proteins, that aggregate with a highly abnormal cross B sheet confirmation [1,2]. Amyloid deposition is associated with disruption of tissue structure and disturbance of organ function, which if left unchecked, invariably leads to organ failure [3,4]. Cardiac amyloidosis (CA) classically manifests with restrictive cardiomyopathy, is a fatal condition, associated with significant morbidity and is often challenging to diagnose. In the majority of cases the amyloid fibril precursor protein is either a monoclonal immunoglobulin light chain (AL) [5] or transthyretin (ATTR); the latter unmutated, wild-type transthyretin amyloidosis (wtATTR), or mutated, variant transthyretin amyloidosis (vATTR) [6–8].