Triple-trouble cardiomyopathy: myocardial infarction, diastolic heart failure, and conduction defects due to amyloidosis associated with multiple myeloma

Gościniak, Piotr; Larysz, Barbara; Józwa, Robert

doi:10.5603/kp.2013.0109

Cited by 3 publications

(3 citation statements)

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“…Chest X-ray findings described in the literature include moderate cardiac enlargement, particularly of the RA, RV, and pulmonary artery, along with increased pulmonary vascularity [ 25 , 26 , 27 , 44 ].…”

Section: Resultsmentioning

confidence: 99%

Congenital Gerbode Defect: A Left Ventricular to Right Atrial Shunt—State-of-the-Art Review of Its General Data, Diagnostic Modalities, and Treatment Strategies

Winter,

Strizek,

Recker

2024

JCDD

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The congenital Gerbode defect is defined as an abnormal communication between the left ventricle and the right atrium. This review aimed to summarize existing evidence, shed light on the clinical implications, and identify knowledge gaps. The systematic literature search was conducted in the PubMed and Google Scholar medical databases using specifically selected keywords. The inclusion of each publication was assessed according to predefined eligibility criteria based on the PICOM (Population, Phenomenon of Interest, Context, Methodology) schema. Titles and abstracts were screened independently by two authors. Available full-text versions of included publications were reviewed and relevant information was extracted. A total of 78 reports were included. The compilation of all congenital Gerbode defect cases described in the literature revealed a variety of clinical presentations comprising dyspnea, palpitations, growth retardation, and asymptomatology. A suitable multimodal diagnostic approach for newborns consists of auscultation, TTE, and optionally TEE and MRI. Because of its rarity, diversity of findings, unknown pathophysiology, and similarity to more common cardiac diseases, the diagnostic challenge remains significant. To prevent untreated long-term sequelae, early individualized treatment is recommended. Surgical defect closure is preferred to device closure for evidence reasons, although major developments are currently taking place. In conclusion, the congenital Gerbode defect provides a diagnostic challenge for pediatricians to allow early diagnosis and intervention in order to improve patients’ quality of life.

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Section: Resultsmentioning

confidence: 99%

Congenital Gerbode Defect: A Left Ventricular to Right Atrial Shunt—State-of-the-Art Review of Its General Data, Diagnostic Modalities, and Treatment Strategies

Winter,

Strizek,

Recker

2024

JCDD

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“…Unfortunately, the clinical symptoms of both forms of amyloidosis (AL-CA and ATTR-CA) are not characteristic of this disease, and an early diagnosis is therefore difficult, requires consultations with many specialists and takes a long time, 27 often until echocardiography is performed by an experienced cardiologist. 28 The most common signs and symptoms of all forms of amyloidosis are listed in Table 1.…”

Section: Clinical Presentationmentioning

confidence: 99%

Updates for the diagnosis and management of cardiac amyloidosis

et al. 2022

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A substantial increase in the interest in transthyretin cardiac amyloidosis (ATTR-CA) is a result of the constantly growing number of patients, the use of clear diagnostic protocols and the availability of the first selective drug for these patients. This has also raised the awareness of the disease among physicians of all specialties. The topic is particularly relevant to cardiologists, who use non-invasive multimodal imaging in their daily practice.The differential diagnosis of the causes of myocardial hypertrophy includes arterial hypertension, hypertrophic cardiomyopathy, aortic stenosis (AS), athletic heart syndrome, Fabry disease, and cardiac amyloidosis (CA). It turns out that in patients with myocardial hypertrophy >15 mm, amyloidosis is the most common cause of left ventricular (LV) hypertrophy. In parallel, CA is one of the most common infiltrative diseases leading to a clinical picture that may mimic heart failure with preserved ejection fraction (HFpEF).The accumulation of amyloid in the extracellular space impairs the diastolic function of the myocardium, which is observed as the restrictive cardiomyopathy phenotype. In advanced cases, the LV systolic function is also impaired. Moreover, protein deposits contribute to the disturbances of calcium metabolism and cell metabolism as well as to cardiotoxicity, leading to edema and damage to cardiomyocytes.

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“…Low voltage in the EKG is seen in more than 50% of the patients with heart involvement [26]. The presentation can also include syncope, arrhythmia, sudden cardiac death, and rarely amyloid deposition in the coronary arterioles that can present as myocardial infarction [27, 28] or cardiogenic shock [29].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

When to Suspect a Diagnosis of Amyloidosis

Vaxman

Gertz

2020

Acta Haematol

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Amyloidosis is a group of complex diseases caused by extracellular deposition of pathological insoluble fibrillary protein in organs and tissues and may result in severe organ dysfunction. Despite the etiological heterogeneity of systemic amyloidosis, the clinical manifestations of the different forms of amyloidosis largely overlap and depend upon the effected organ. The signs and symptoms that should raise suspicion for the potential diagnosis of amyloidosis are usually nonspecific; therefore, establishing the diagnosis is difficult, and early diagnosis requires clinical suspicion. Light chain (AL) amyloidosis may present with highly specific signs such as macroglossia and periorbital purpura, but these signs are insensitive. Amyloidosis is still underdiagnosed, even though treatments are now available and are effective in improving patient's survival and quality of life. Cardiac amyloidosis is the major determinant of survival, and the earlier it is detected the better the survival. All MGUS patients should be routinely screened for AL amyloidosis by a focused history and physical examination and routine assessment of urine albumin. The aim of this review is to provide clinicians with knowledge about the signs and symptoms that raise the suspicion of amyloidosis, bearing in mind the importance of early diagnosis of this disease.

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Triple-trouble cardiomyopathy: myocardial infarction, diastolic heart failure, and conduction defects due to amyloidosis associated with multiple myeloma

Cited by 3 publications

References 0 publications

Congenital Gerbode Defect: A Left Ventricular to Right Atrial Shunt—State-of-the-Art Review of Its General Data, Diagnostic Modalities, and Treatment Strategies

Congenital Gerbode Defect: A Left Ventricular to Right Atrial Shunt—State-of-the-Art Review of Its General Data, Diagnostic Modalities, and Treatment Strategies

Updates for the diagnosis and management of cardiac amyloidosis

When to Suspect a Diagnosis of Amyloidosis

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