Home inotrope therapy in chronic stimulant-induced cardiomyopathy: a case series
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PROVIDER: S-EPMC11336994 | biostudies-literature | 2024 Aug
REPOSITORIES: biostudies-literature
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Project description:BackgroundCardiotoxicity presenting as cardiomyopathy is a common side effect in cancer treatment especially with anthracyclines. The role of genetic predisposition is still being investigated.Case summaryFour unrelated patients with a familial burden for cardiac disease, who developed cardiomyopathy after anthracycline treatment are presented. Case 1 received chemotherapy for breast cancer and developed a dilated left ventricle just after treatment. Her father had died unexpectedly while being screened for heart transplant. Case 2 was known with a family history of sudden cardiac death prior to her breast cancer diagnosis. She received anthracycline-containing chemotherapy treatment twice in 5 years due to recurrence of breast cancer. During that period, two brothers developed a cardiomyopathy. Eighteen years later, a genetic predisposition for cardiomyopathy was ascertained and at screening an asymptomatic non-ischaemic cardiomyopathy was established. Case 3 was diagnosed with a dilated cardiomyopathy 1 year after chemotherapy treatment for breast cancer. Her mother had developed a dilated cardiomyopathy several years before. Case 4 received chemotherapy treatment for Non-Hodgkin's lymphoma and developed dilated cardiomyopathy 1 year later. His brother died from congestive heart failure which he developed after chemotherapy for Non-Hodgkin's lymphoma and a grandmother had died suddenly during child delivery. In all four cases, genetic screening showed (likely) pathogenic variants in cardiomyopathy-associated genes.DiscussionCurrent guidelines recommend cardiac evaluation in cancer patients receiving chemotherapy based on the presence of cardiovascular risk factors at the start of treatment. This series emphasizes the importance of including a thorough family history in this process.
| S-EPMC8536866 | biostudies-literature
Project description:BackgroundHis-bundle pacing (HBP) alone may become an alternative to conventional cardiac resynchronization therapy (CRT) utilizing right ventricular apical (RVA) and left ventricular (LV) pacing (BiVRVA+LV) in selected patients, but the effects of CRT utilizing HBP and LV pacing (BiVHB+LV) on cardiac resynchronization and heart failure (HF) are unclear.Case summaryWe presented two patients with inotrope-dependent end-stage HF in whom the upgrade from conventional BiVRVA+LV to BiVHB+LV pacing by the addition of a lead for HBP improved their HF status. Patient 1 was a 32-year-old man with lamin A/C cardiomyopathy, atrial fibrillation, and complete atrioventricular (AV) block. Patient 2 was a 70-year-old man with ischaemic cardiomyopathy complicated by AV block and worsening of HF resulting from ablation for ventricular tachycardia storm. The HF status of both patients improved dramatically following the upgrade from BiVRVA+LV to BiVHB+LV pacing.DiscussionEnd-stage HF patients suffer from diffuse intraventricular conduction defect not only in the LV but also in the right ventricle (RV). The resulting dyssynchrony may not be sufficiently corrected by conventional BiVRVA+LV pacing or HBP alone. Right ventricular apical pacing itself may also impair RV synchrony. An upgrade to BiVHB+LV pacing could be beneficial in patients who become non-responsive to conventional BiV pacing as the His-Purkinje conduction defect progresses.
| S-EPMC7793217 | biostudies-literature
Project description:BackgroundHypertrophic cardiomyopathy in identical twins is rare. Cases of hypertrophic cardiomyopathy with homogenous and heterogeneous phenotypes have been described in the literature.Case summaryWe report a pair of monozygotic twins (Twin A and Twin B) with identical morphological expression of hypertrophic cardiomyopathy. On initial evaluation, both twins had resting left ventricular outflow tract obstruction, Grade II diastolic dysfunction, and New York Heart Association (NYHA) Class II symptoms, but they had a different clinical course afterward. Twin A progressed from NYHA Class II to Class III with a high left ventricular outflow tract pressure gradient that was unresponsive to medical treatment and required alcohol septal ablation. Twin B responded very well to medical treatment. Both patients had no risk factors for sudden cardiac death, and neither required an implantable cardioverter defibrillator.DiscussionThe morphology of hypertrophic cardiomyopathy has a strong genetic basis, but epigenetic factors may affect disease expression.
| S-EPMC9793770 | biostudies-literature
Project description:BackgroundPeripartum cardiomyopathy (PPCM) is usually characterized by overt heart failure, but other clinical scenarios are possible, sometimes making the diagnosis challenging.Case summaryWe report a case series of four patients with PPCM. The first patient presented with acute heart failure due to left ventricular (LV) systolic dysfunction. Following medical treatment, LV function recovered completely at 1 month. The second patient had systemic and pulmonary thromboembolism, secondary to severe biventricular dysfunction with biventricular thrombi. The third patient presented with myocardial infarction with non-obstructed coronary arteries and evidence of an aneurysm of the mid-anterolateral LV wall. The fourth patient, diagnosed with PPCM 11 years earlier, presented with sustained ventricular tachycardia. A repeat cardiac magnetic resonance, compared to the previous one performed 11 years earlier, showed an enlarged LV aneurysm in the mid-LV anterolateral wall with worsened global LV function.DiscussionPeripartum cardiomyopathy may have different clinical presentations. Attentive clinical evaluation and multimodality imaging can provide precise diagnostic and prognostic information.
| S-EPMC7954255 | biostudies-literature
Project description:BackgroundSaw-tooth cardiomyopathy (STC) is an unusual type of left ventricular dysplasia. To our knowledge, six cases have been reported in the literature. Two new cases are presented with a review of all the case reports that have been published.Case summaryTwo patients with STC were examined. The first one was a 69-year-old woman with shortness of breath on mild exertion and chest pain, and the second was a 49-year-old man with a history of myocardial infarction who required stent implantation and is now asymptomatic. Both patients revealed findings of STC in the cardiac computed tomography (CT).DiscussionWhen analysing the cases and comparing them to the ones reported in the literature; STC is a generally benign heart disease, although the clinical spectrum can range from asymptomatic to heart failure. Imaging studies such as cardiac magnetic resonance and cardiac CT are essential for the diagnosis.
| S-EPMC8849121 | biostudies-literature
Project description: Not available
| S-EPMC3904030 | biostudies-literature
Project description:BACKGROUND:The sepsis-induced immunodepression contributes to impaired clinical outcomes of various stress conditions. This syndrome is well documented and characterized by attenuated function of innate and adaptive immune cells. Several pharmacological interventions aimed to restore the immune response are emerging of which interferon-gamma (IFNγ) is one. It is of paramount relevance to obtain clinical information on optimal timing of the IFNγ-treatment, -tolerance, -effectiveness and outcome before performing a RCT. We describe the effects of IFNγ in a cohort of 18 adult and 2 pediatric sepsis patients. METHODS:In this open-label prospective multi-center case-series, IFNγ treatment was initiated in patients selected on clinical and immunological criteria early (< 4 days) or late (> 7 days) following the onset of sepsis. The data collected in 18 adults and 2 liver transplanted pediatric patients were: clinical scores, monocyte expression of HLA-DR (flow cytometry), lymphocyte immune-phenotyping (flow cytometry), IL-6 and IL-10 plasma levels (ELISA), bacterial cultures, disease severity, and mortality. RESULTS:In 15 out of 18 patients IFNγ treatment was associated with an increase of median HLA-DR expression from 2666 [IQ 1547; 4991] to 12,451 [IQ 4166; 19,707], while the absolute number of lymphocyte subpopulations were not affected, except for the decrease number of NK cells 94.5 [23; 136] to 32.5 [13; 90.8] (0.0625)]. Plasma levels of IL-6 464 [201-770] to 108 (89-140) ng/mL (p = 0.04) and IL-10 from IL-10 from 29 [12-59] to 9 [1-15] pg/mL decreased significantly. Three patients who received IFNγ early after ICU admission (<4 days) died. The other patients had a rapid clinical improvement assessed by the SOFA score and bacterial cultures that were repeatedly positive became negative. The 2 pediatric cases improved rapidly, but 1 died for hemorrhagic complication. CONCLUSION:Guided by clinical and immunological monitoring, adjunctive immunotherapy with IFNγ appears well-tolerated in our cases and improves immune host defense in sepsis induced immuno suppression. Randomized clinical studies to assess its potential clinical benefit are warranted.
| S-EPMC6833157 | biostudies-literature
Project description:The diagnosis and treatment of new-onset systolic dysfunction can be challenging, particularly in patients presenting with cardiogenic shock. We present a case of a young African-American man who was admitted for cardiogenic shock following an admission a month earlier for treatment- resistant psychosis. He was diagnosed with medication-induced cardiomyopathy, which resolved with a remarkable recovery of his systolic function after discontinuation of the culprit medication, clozapine.
| S-EPMC11526494 | biostudies-literature
Project description:Patients with restrictive cardiomyopathy due to Fabry disease are often deemed ineligible for left ventricular assist device (LVAD) support due to the risk of suction events with a small LV cavity. We describe the first case series of LVAD support for Fabry disease. LVAD therapy can improve survival, quality of life, and provide clinical stability to start enzyme replacement therapy. Precautions at the time of surgery include rapid treatment of fever to avoid Fabry crises, involvement of a multidisciplinary team, and early initiation of rehabilitation. We describe LVAD support for both bridging and destination therapy.
| S-EPMC7893197 | biostudies-literature
Project description:BackgroundClinical features and imaging presentation of myocarditis can overlap with other inflammatory or arrhythmogenic cardiomyopathies. Desmoplakin (DSP) is an important structural cardiac protein. Mutations in the DSP gene are associated with a variant of arrhythmogenic right ventricular cardiomyopathy (ARVC). Interestingly, this distinct genetic cardiomyopathy can also present with a myocardial inflammation and fibrosis pattern that may mimic other forms of myocarditis including viral myocarditis, which can raise a clinical challenge. We report two cases of DSP cardiomyopathy, which were initially thought to represent coronavirus disease of 2019 (COVID-19) myocarditis.Case summaryFirst patient is a 21-your-old woman with no past medical history but family history of presumed 'viral myocarditis' and ventricular tachycardia in her brother. She presented with acute chest pain and elevated cardiac enzymes. She tested positive for COVID-19 and given the suspicion for possible COVID-19 related acute myocarditis, cardiac magnetic resonance imaging obtained and revealed regional wall motion abnormalities, several areas of subepicardial and pericardial late gadolinium enhancement (LGE). Ambulatory cardiac monitoring showed runs of non-sustained ventricular tachycardia and considering her family history of arrhythmogenic myocarditis, genetic testing was performed that was positive for a likely pathogenic heterozygous mutation of DSP gene. She declined the recommended implantable cardioverter defibrillator (ICD).Second patient is a 34-year-old physician with no significant past medical history who works at a COVID-19 unit and presented with syncope and was found to have ventricular tachycardia. Echocardiogram revealed severely dilated left ventricle and globally depressed systolic function with left ventricular ejection fraction of 20%. Coronary computed tomography angiography showed no evidence of coronary atherosclerosis. Cardiac magnetic resonance imaging revealed several areas of mid myocardial and pericardial LGE. Subcutaneous ICD was implanted and an endomyocardial biopsy had evidence of lymphocytic myocarditis and adipose tissue infiltration of the myocardium. Genetic testing revealed pathogenic heterozygous DSP mutation. He underwent epicardial ablation for the episodes of ventricular tachycardia despite medical therapy. He was able to return to work and has not had any further episodes of arrhythmia.ConclusionMutations in the DSP gene are associated with left dominant arrhythmogenic cardiomyopathy, which is a variant of ARVC. Beside left ventricular systolic dysfunction and ventricular tachyarrhythmias, carriers of these mutations may present with episodes of chest pain associated with elevated cardiac enzymes and cardiac imaging findings indistinguishable from other forms of acute myocarditis including viral myocarditis. Currently, there are no guidelines for diagnosis and treatment of this entity.
| S-EPMC9384800 | biostudies-literature