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Incessant ectopic atrial tachycardia and tachycardia-related cardiomyopathy: therapeutic options and potential for cure

Shah RP, Kam RM, Teo WS

Ann Acad Med Singap, 1999 Nov;28(6):871-4.
PMID: 10672407

Incessant ectopic atrial tachycardia (IEAT) is a rare cause of cardiomyopathy. Cardiomyopathy is reversible by curative ablation using surgery or radiofrequency current. We report our experience with 5 patients with IEAT. Three patients presented with palpitations and were diagnosed to have paroxysmal supraventricular tachycardia (2 patients) and atrial flutter with 1:1 conduction (1 patient), but 2 presented insidiously with congestive cardiac failure. All the initial echocardiograms showed left ventricular dysfunction. The patients underwent electrophysiological studies which confirmed the diagnosis of IEAT. The first patient had surgical cryoablation and the other patients had successful radiofrequency catheter ablation. Follow-up for 2 to 7 years has shown no recurrences. All patients had significant improvement in left ventricular function on echocardiography. In conclusion, curative ablation by surgery or radiofrequency current is safe and effective. Because of its low morbidity, radiofrequency catheter ablation should be the treatment of choice for IEAT, especially if complicated by tachycardia-related cardiomyopathy.

Matched MeSH terms: Cardiomyopathies/etiology*
Acute myopathy after status asthmaticus: steroids, myorelaxants or carbon dioxide?

Goh AY, Chan PW

Respirology, 1999 Mar;4(1):97-9.
PMID: 10339738

Acute myopathy complicating treatment of status asthmaticus has been increasingly recognized since its original description in 1977. We report a case of an 11-year-old boy with severe asthma requiring mechanical ventilation. He was given high doses of parenteral steroids and neuromuscular blockade with non-depolarizing agents in order to achieve controlled hypoventilation with an ensuing hypercapnoea. He developed rhabdomyolysis with elevated creatinine kinase and renal impairment secondary to myoglobinuria. Electrophysiological studies revealed myopathic abnormalities. The aetiology for this myopathy appears to be related to therapy with parenteral steroids, muscle-relaxant agents and respiratory acidosis. Patients treated with steroids and neuromuscular blocking agents should be regularly monitored for development of myopathy.

Matched MeSH terms: Cardiomyopathies/etiology*
Diastolic dysfunction in patients with liver cirrhosis: A short-term, observational study at a Malaysian hospital

Abdul Aziz KA, Draman N, Wan Isa WYH, Mustaffa N

Med J Malaysia, 2020 07;75(4):396-399.
PMID: 32724001

Cirrhotic cardiomyopathy is a recognised complication of liver cirrhosis and predicts poor outcomes. Detection of diastolic dysfunction, an early indicator of left ventricular dysfunction can help identify those patients at risk of disease progression. In our study we showed that there was a high prevalence of diastolic dysfunction amongst patients with liver cirrhosis at our outpatient clinic, with the majority being Child-Pugh A/low MELD score. Multiple regression analysis indicated that age and sodium levels were significantly associated with the presence of diastolic dysfunction. This further reinforces the importance of dietary sodium restriction amongst patients with liver cirrhosis.

Matched MeSH terms: Cardiomyopathies/etiology*
Cardiorespiratory Progression Over 5 Years and Role of Corticosteroids in Duchenne Muscular Dystrophy: A Single-Site Retrospective Longitudinal Study

Trucco F, Domingos JP, Tay CG, Ridout D, Maresh K, Munot P, et al.

Chest, 2020 10;158(4):1606-1616.
PMID: 32387519 DOI: 10.1016/j.chest.2020.04.043

BACKGROUND: Corticosteroids (CSs) have prolonged survival and respiratory function in boys with Duchenne muscular dystrophy (DMD) when compared with CSs-naïve boys.
RESEARCH QUESTION: The differential impact of frequently used CSs and their regimens on long-term (> 5 years) cardiorespiratory progression in children with DMD is unknown.
STUDY DESIGN AND METHODS: This was a retrospective longitudinal study including children with DMD followed at Dubowitz Neuromuscular Centre, Great Ormond Street Hospital London, England, from May 2000 to June 2017. Patients enrolled in any interventional clinical trials were excluded. We collected patients' anthropometrics and respiratory (FVC, FVC % predicted and absolute FVC, and noninvasive ventilation requirement [NIV]) and cardiac (left ventricular shortening function [LVFS%]) function. CSs-naïve patients had never received CSs. Patients who were treated with CSs took either deflazacort or prednisolone, daily or intermittently (10 days on/10 days off) for > 1 month. Average longitudinal models were fitted for yearly respiratory (FVC % predicted) and cardiac (LVFS%) progression. A time-to-event analysis to FVC % predicted < 50%, NIV start, and cardiomyopathy (LVFS% < 28%) was performed in CS-treated (daily and intermittent) vs CS-naïve patients.
RESULTS: There were 270 patients, with a mean age at baseline of 6.2 ± 2.3 years. The median follow-up time was 5.6 ± 3.5 years. At baseline, 263 patients were ambulant. Sixty-six patients were treated with CSs daily, 182 patients underwent CSs intermittent > 60% treatment, and 22 were CS-naïve patients. Yearly FVC % predicted declined similarly from 9 years (5.9% and 6.9% per year, respectively; P = .27) in the CSs-daily and CSs-intermittent groups. The CSs-daily group declined from a higher FVC % predicted than the CSs-intermittent group (P < .05), and both reached FVC % predicted < 50% and NIV requirement at a similar age, > 2 years later than the CS-naïve group. LVFS% declined by 0.53% per year in the CSs-treated group irrespective of the CSs regimen, significantly slower (P < .01) than the CSs-naïve group progressing by 1.17% per year. The age at cardiomyopathy was 16.6 years in the CSs-treated group (P < .05) irrespective of regimen and 13.9 years in the CSs-naïve group.
INTERPRETATION: CSs irrespective of the regimen significantly improved respiratory function and delayed NIV requirement and cardiomyopathy.

Matched MeSH terms: Cardiomyopathies/etiology*
Isolated atrial amyloidosis: a clinicopathologic study indicating increased prevalence in chronic heart disease

Looi LM

Hum Pathol, 1993 Jun;24(6):602-7.
PMID: 8505038

Congo red screening of 211 consecutive cardiac biopsy specimens obtained during cardiac surgery from 167 patients revealed 26 (16%) instances of isolated atrial amyloidosis (IAA). The ages of IAA-positive patients ranged from 25 to 52 years (mean age, 39 years). Twenty-three (88%) IAA-positive biopsy specimens were from patients with chronic rheumatic heart disease (CRHD) while three (12%) were from patients with an atrial septal defect (ASD). The prevalence of IAA in the CRHD patients was 23%, appreciably higher than that in the ASD patients (15%) and in other patients with atrial biopsies. The prevalence of IAA in both CRHD and ASD patients was significantly higher (P < .001) than in controls. Controls consisted of 247 healthy adults who were autopsied after traumatic deaths, with an age range of 18 to 89 years (mean age, 38 years). Only seven (3%) control subjects were IAA positive; all were over 40 years of age. Isolated atrial amyloidosis deposits were permanganate resistant and immunohistochemically positive for human amyloid P (AP) protein and negative for human amyloid-associated (AA) protein and immunoglobulin light chains. They were observed as fine congophilic and birefringent deposits in intramyocardial vessel walls, along the myocardial sarcolemma, and in the subendocardium. There was associated myocyte hypertrophy but no atrophy. Electron microscopy demonstrated typical nonbranching amyloid fibrils. It is postulated that stretching of the atria in chronic heart disease results in a raised prevalence of IAA. Recent reports that IAA contains atrial natriuretic peptide, a polypeptide hormone product of atrial myocytes, supports this view.

Matched MeSH terms: Cardiomyopathies/etiology
Fulltext Heart and bile acids - Clinical consequences of altered bile acid metabolism

Vasavan T, Ferraro E, Ibrahim E, Dixon P, Gorelik J, Williamson C

Biochim Biophys Acta Mol Basis Dis, 2018 04;1864(4 Pt B):1345-1355.
PMID: 29317337 DOI: 10.1016/j.bbadis.2017.12.039

Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart. Elevated maternal serum bile acids in intrahepatic cholestasis of pregnancy, a disorder which causes an impaired feto-maternal bile acid gradient, have been associated with fatal fetal arrhythmias. The hydrophobicity of individual bile acids in the serum bile acid pool is of relevance, with relatively lipophilic bile acids having a more harmful effect on the heart. Ursodeoxycholic acid can reverse or protect against these detrimental cardiac effects of elevated bile acids.

Matched MeSH terms: Cardiomyopathies/etiology*