Dilated cardiomyopathy of possible dietary origin in a cat.

An 11-year-old spayed female domestic shorthaired cat was diagnosed with severe dilated cardiomyopathy (DCM) and congestive heart failure. The cat had been eating cat foods that were high in pulses (e.g. peas, lentils, chickpeas). Neither plasma nor whole blood taurine concentrations were deficient. Primary treatment included furosemide, pimobendan, and clopidogrel, and changing to diets that did not contain pulses (a taurine supplements was not administered). The cat's clinical signs improved, high-sensitivity cardiac troponin I concentrations decreased, and echocardiographic measurements stayed relatively stable for over one year after initiating cardiac medications and changing the diet. Ultimately, the cat was euthanized for worsening congestive heart failure 374 days after the diagnosis of DCM. Infectious disease testing during the time of clinical surveillance was negative. Routine histopathology of the heart was unremarkable, but electron microscopy of the left ventricle showed large numbers of mitochondria of variable size and structure. A moderate number of lamellar bodies and autophagic vacuoles also were noted. This case report illustrates an unusual case of a cat with DCM unrelated to taurine deficiency. The relative roles of diet change, cardiac medications, and a dedicated owner are unclear, but this cat's relatively long survival time is similar to that seen after diet change in dogs and cats with DCM eating high-pulse diets.

Hypocalcaemia As A Rare Reversible Cause Of Dilated Cardiomyopathy.

Dilated cardiomyopathy is characterized by dilation and enlargement of one or both ventricles with reduced systolic function. Calcium plays a key role in myocardial contraction. Hypocalcaemia can lead to a decrease in contraction, left ventricular systolic dysfunction, and heart failure with reduced ejection fraction (EF). Hypocalcaemia is a rare reversible cause of dilated cardiomyopathy. The author presents a case who presented with complaints of shortness of breath on exertion, orthopnoea, paroxysmal nocturnal dyspnoea, numbness and crampy muscular pains. He had a high JVP, systolic murmur on auscultation, hepatomegaly, pedal oedema and crackles on chest auscultation. His ECG showed sinus rhythm with prolonged QT interval. His echocardiography showed dilated cardiomyopathy with reduced ejection fraction, moderate mitral regurgitation and mild tricuspid regurgitation. His Calcium levels and PTH levels were both low. He was treated with ionotrophes, diuretics, vitamin D and calcium supplements, including both intravenous and oral. With the correction of calcium levels, he was weaned off the ionotrophic support and his ejection fraction improved. Calcium levels if low should be corrected in patients with dilated cardiomyopathy.

Nonsustained Ventricular Tachycardia Is Independently Associated With Sustained Ventricular Arrhythmias in Nonischemic Dilated Cardiomyopathy.

BACKGROUND: Spontaneous nonsustained ventricular tachycardia (NSVT) on Holter, VT inducibility during electrophysiology study, and late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) have been associated with sustained ventricular arrhythmias (SVAs) in nonischemic dilated cardiomyopathy (DCM). This study aimed to analyze whether these parameters carry independent prognostic value for spontaneous SVA in DCM. METHODS: Between 2011 and 2018, patients with the DCM clinical spectrum and documented SVA, suspected SVA, or considered to be at intermediate or high risk for SVA were enrolled in the prospective Leiden Nonischemic Cardiomyopathy Study. Patients underwent a comprehensive evaluation including 24-hour Holter, LGE-CMR, and electrophysiology study. Holters were assessed for the presence of NSVT (≥3 beats; rate, ≥120 bpm; lasting <30 s) and NSVT characteristics (coupling interval, duration, cycle length, morphology, regularity). Patients were followed at 6 to 12 monthly intervals. RESULTS: Of all 115 patients (age, 59±12 years; 77% men; left ventricular ejection fraction, 33±13%; history of SVA, 36%; LGE in 63%; median LGE mass, 13 g; interquartile range, 8-23 g), 62 (54%) had NSVT on Holter, and sustained monomorphic VT was inducible in 34 of 114 patients (30%). NSVT was not associated with LGE on CMR or VT inducibility during electrophysiology study nor were its features (all P>0.05). During 4.0±1.8 years of follow-up, SVA occurred in 39 patients (34%). NSVT (HR, 4.47 [95% CI, 1.87-10.72]; P=0.001) and VT inducibility (HR, 3.08 [95% CI, 1.08-8.81]; P=0.036) were independently associated with SVA during follow-up. A bivariable model including only noninvasively acquired parameters also allowed identification of a high-risk subgroup (ie, those with both NSVT and LGE on CMR). The findings remained similar when only patients without prior SVA were included. CONCLUSIONS: In patients with DCM, NSVT on Holter and VT inducibility during electrophysiology study predict SVA during follow-up independent of LGE on CMR. NSVTs may serve as an initiator, and sustained VT inducibility indicates the presence of the substrate for SVA in DCM. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01940081.

Novel dilated cardiomyopathy associated to Calreticulin and Myo7A gene mutation in Usher syndrome.

We report a novel cardiomyopathy associated to Usher syndrome and related to combined mutation of MYO7A and Calreticulin genes. A 37-year-old man with deafness and vision impairment because of retinitis pigmentosa since childhood and a MYO7A gene mutation suggesting Usher syndrome, developed a dilated cardiomyopathy with ventricular tachyarrhythmias and recurrent syncope. At magnetic resonance cardiomyopathy was characterized by left ventricular dilatation with hypo-contractility and mitral prolapse with valve regurgitation. At left ventricular endomyocardial biopsy, it was documented cardiomyocyte disconnection because of cytoskeletal disorganization of cell-to-cell contacts, including intercalated discs, and mitochondrial damage and dysfunction with significant reduction of adenosine triphosphate production in patient cultured fibroblasts. At an extensive analysis by next-generation-sequencing of 4183 genes potentially related to the cardiomyopathy a pathogenic mutation of calreticulin was found. The cardiomyopathy appeared to be functionally and electrically stabilized by a combination therapy including carvedilol and amiodarone at a follow-up of 18 months.

Management of nonischemic-dilated cardiomyopathies in clinical practice: a position paper of the working group on myocardial and pericardial diseases of Italian Society of Cardiology.

: Nonischemic-dilated cardiomyopathy (NIDCM) is an entity that gathers extremely heterogeneous diseases. This awareness, although leading to continuous improvement in survival, has increased the complexity of NIDCM patients' management. Even though the endorsed 'red-flags' approach helps clinicians in pursuing an accurate etiological definition in clinical practice, it is not clear when and how peripheral centers should interact with referral centers with specific expertise in challenging scenarios (e.g. postmyocarditis and genetically determined dilated cardiomyopathy) and with easier access to second-line diagnostic tools and therapies. This position paper will summarize each step in NIDCM management, highlighting the multiple interactions between peripheral and referral centers, from first-line diagnostic workup and therapy to advanced heart failure management and long-term follow-up.

Hypocalcaemic cardiomyopathy: a description of two cases and a literature review.

Hypocalcaemic cardiomyopathy is a rare form of dilated cardiomyopathy. The authors here present two cases in which symptomatic dilated cardiomyopathy was the result of severe hypocalcaemia. First, we report about a 26-year-old woman with primary hypoparathyroidism and then about a 74-year-old man with secondary hypoparathyroidism following a thyroidectomy. In both cases, the left ventricular systolic function improved after calcium supplementation. In the first case, a lack of compliance led to a repeated decrease of both serum calcium level and left ventricular systolic function. The authors also present a comprehensive summary of all cases of hypocalcaemic dilated cardiomyopathy that have been described in literature to date. The mean age of the affected patients was 48.3 years, of which 62% were female patients. The most common causes of hypocalcaemic cardiomyopathy are primary hypoparathyroidism (50%) and post-thyroidectomy hypoparathyroidism (26%). In the post-thyroidectomy subgroup, the median time for the development of hypocalcaemic cardiomyopathy is 10 years (range: 1.5 months to 36 years). Hypocalcaemic cardiomyopathy leads to heart failure with reduced ejection fraction in 87% of patients. Generally, the most common complications of hypoparathyroidism and/or hypocalcaemia are cerebral calcifications, cognitive deficit, and cataracts. Once calcium supplementation is administered, the disease has a good prognosis and, in most individuals, a significant improvement (21%) or even normalization (74%) of the left ventricular systolic function occurs.

Vitamin D deficiency cardiomyopathy in Scotland: a retrospective review of the last decade.

OBJECTIVE: To determine the incidence, demography and prognosis of vitamin D deficiency dilated cardiomyopathy (DCM) in Scotland over the last decade. STUDY DESIGN: A retrospective review of cases of vitamin D deficiency DCM presenting to a national paediatric cardiac centre between 1 January 2008 and 1 January 2018. The departmental database and electronic and paper case notes were used to identify patients and extract data. RESULTS: Six patients were identified (three male), three of whom were Caucasian. Median age at presentation was 206 days (range 2-268.) All six patients had high serum parathyroid hormone levels (median 45 pmol/L, range 27-120 pmol/L), a sensitive marker of total body calcium deprivation secondary to vitamin D deficiency. All patients demonstrated clinical and echocardiographic improvement following high dose vitamin D treatment. No patients required cardiac transplant, and only one patient required extracorporeal life support as a bridge to recovery. After an initial improvement, one child died at 5 months as a result of respiratory infection. Three patients lived within some of the most deprived areas in Scotland. CONCLUSIONS: This case series demonstrates a previously unreported demographic in Scotland, as 50% of cases presented in Caucasian children. Although vitamin D deficiency DCM is relatively rare, it is wholly preventable. Our study confirms that vitamin D deficiency cardiomyopathy is reversible with prompt identification and supplementation. The current implementation of public health policy in the UK is failing to prevent children from developing the most severe manifestation of vitamin D deficiency.

Dilated cardiomyopathy in children: moving beyond traditional pharmacologic therapy.

PURPOSE OF REVIEW: Dilated cardiomyopathy (DCM) is a rare myocardial disorder characterized by a dilated left ventricle and systolic dysfunction. Globally, it affects around 1 in every 100 000 children. The prognosis is generally poor, with 40% either failing traditional medical therapy within the first 2 years or requiring a heart transplant. This article will address the basic cause, epidemiology, pathobiology, and historical treatment approach of DCM and introduce novel contemporary medical and surgical strategies that may reduce the need for heart transplantation. RECENT FINDINGS: In the last 15 years, there has been a significant emphasis on identifying alternative treatment strategies in managing the child with a DCM and heart failure symptoms. New therapies have evolved to help bridge these critically ill children to transplant or have these therapeutic modalities serve as end-points in themselves. Thus subsequently, we will highlight contemporary as well as novel medical and procedural therapies that are being used for the treatment of pediatric DCM. SUMMARY: The child with a DCM and severe left ventricular dysfunction has a number of options available beyond simple diuretics and afterload reduction. Novel pacing strategies and mechanical assist device may provide not only a more stable clinical bridge environment but also may actually serve as an endpoint itself.

Hypocalcemic cardiomyopathy: a rare presenting manifestation of hypoparathyroidism.

Hypoparathyroidism patients present with features of hypocalcemia like carpopedal spasm, numbness and paresthesias but hypocalcemic cardiomyopathy leading to congestive heart failure (CHF) is a rare presentation. We present here a case of 55-year-old Asian man who was a known case of dilated cardiomyopathy for 6 months, presented with the chief complaints of shortness of breath on exertion and decreased urine output. On general physical examination, features suggestive of CHF were seen. Chvostek and Trousseau's sign was positive. The patient had a history of cataract surgery of both eyes 15 years ago. Further investigations revealed hypocalcemia. Echo showed severe global hypokinesia of left ventricle with left ventricle ejection fraction 15%. This CHF was refractory to conventional treatment, though, with calcium supplementation, the patient improved symptomatically. On follow-up after 3 months, an improvement was seen in the echocardiographic parameters with ejection fraction improving to 25%.

Multiple ICD shocks in a patient with dilated cardiomyopathy: What is the mechanism?

We present the case of a 45-year-old male patient with dilated cardiomyopathy who suffers from multiple implantable cardioverter defibrillator (ICD) shocks. The analysis of the ICD tracing and the electrophysiological study allows to conclude that bundle branch re-entrant tachycardia is the most likely diagnosis, even in absence of conduction abnormalities in his basal electrocardiography.

Occurrence of nutritional hypocalcaemic rickets-related dilated cardiomyopathy in a child with concomitant rickets and infantile-onset Pompe disease.

Infantile-onset Pompe disease is a lysosomal storage disorder characterised with hypertrophic cardiomyopathy, respiratory insufficiency, and hypotonia. Dilated cardiomyopathy is an extremely rare and curable complication of nutritional hypocalcaemic rickets. A 3-month-old female infant was referred to our paediatric ICU with a 4-day history of fatigue, tachypnoea, tachycardia, hypoxia, and respiratory failure. According to the laboratory, radiology, and echocardiography findings, she was first diagnosed with nutritional hypocalcaemic rickets-related dilated cardiomyopathy, but vitamin D and elementary calcium supplementation unmasked the underlying infantile-onset Pompe disease. Nutritional hypocalcaemic rickets and infantile-onset Pompe disease must always be kept in mind among the causes of concomitant dilated cardiomyopathy and hypertrophic cardiomyopathy.

Taurine deficiency and dilated cardiomyopathy in golden retrievers fed commercial diets.

INTRODUCTION: Golden retrievers are over-represented in cases of taurine-deficient dilated cardiomyopathy and recently a surge in cases has prompted further investigation. OBJECTIVE: To describe the clinical, dietary, and echocardiographic features in golden retrievers diagnosed with taurine deficiency and dilated cardiomyopathy, and to determine specific dietary associations. A second aim was to determine the whole blood taurine concentrations in a representative sample of healthy golden retrievers. ANIMALS: Twenty-four client-owned golden retrievers with documented taurine deficiency and dilated cardiomyopathy and 52 healthy client-owned golden retrievers. METHODS: In this multicenter prospective observational study, baseline and follow-up echocardiographic data, complete diet and medical histories, and whole blood, plasma, or serum taurine concentrations were obtained. Baseline and follow-up echocardiographic data were compared. Associations were evaluated between specific diets and taurine deficiency or congestive heart failure. The prevalence of low whole blood taurine concentrations in the healthy golden retrievers was calculated. RESULTS: Twenty-three of 24 dogs diagnosed with taurine deficiency and dilated cardiomyopathy were fed diets that were either grain-free, legume-rich, or a combination of these factors. None of these diets were feeding trial tested using Association of American Feed Control Officials (AAFCO) procedures. Twenty-three of 24 dogs had significant improvement in their echocardiographic parameters and normalization of taurine concentrations following diet change and taurine supplementation. Nine of 11 dogs diagnosed with congestive heart failure (CHF) had resolution of their congestion at follow-up with five no longer requiring diuretic therapy and four tolerating diuretic dose reduction by >50%. CONCLUSIONS: Certain diets and diet characteristics were associated with the development of taurine deficiency. Taurine deficiency and dilated cardiomyopathy in golden retrievers is likely multifactorial, including a combination of dietary, metabolic, and genetic factors.

Troubleshooting electromagnetic interference in a patient with centrifugal flow left ventricular assist device and subcutaneous implantable cardioverter defibrillator.

A 25-year-old man with severe nonischemic dilated cardiomyopathy underwent subcutaneous implantable cardioverter defibrillator (S-ICD) implant and subsequently underwent HeartWare ventricular assist device (HVAD) placement. Postoperative interrogation revealed both primary and secondary S-ICD vectors inappropriately regarded sinus rhythm as "noise," and the alternate vector significantly undersensed sinus rhythm. The S-ICD was reinterrogated using high-resolution capture to visually confirm EMI with a dominant frequency in both the primary and secondary vectors of 46.67 Hz that fell within the S-ICD operational range of 9-60 Hz. The 46.67 Hz frequency correlated with the HVAD operational speed of 2,800 RPM. The HVAD pump speed was increased from 2,800 to 3,000 RPM, resulting in a dominant frequency of 50 Hz. The notch filter is nonprogrammable in S-ICDs. However, the built-in filter is 50 Hz for countries in European time zones as opposed to 60 Hz in US time zones due to differences in the anticipated noise from electrical sources within each continent. Thus, the S-ICD time zone was reprogrammed from EST to GMT, which reduced the notch filter from 60  to 50 Hz, resulting in S-ICD successfully eliminating EMI when the patient was in a supine position. The EMI interference was still intermittently present in the upright patient position. This case demonstrates the utility of high-resolution electrogram capture to identify the source and frequency of EMI in S-ICD and offers a potential avenue to troubleshoot dominant frequency oversensing by changing the device time zone.

Diagnóstico ecocardiográfico e tratamento de cardiomiopatia dilatada em tamanduá- bandeira ( Myrmecophaga trydactila ) mantido em cativeiro: relato de caso / Echocardiographic diagnosis and treatment of dilated cardiomyopathy in captive giant anteater (Myrmecophaga trydactila): case report

A cardiomiopatia dilatada é uma doença de caráter crônico, que compromete a função cardíaca, resultando em desequilíbrio da circulação sanguínea e da homeostase corporal do animal. Este relato apresenta a evolução do quadro clínico e o tratamento de cardiomiopatia dilatada em um exemplar cativo de tamanduá-bandeira. O animal apresentou quadro clínico de insuficiência cardíaca e foi submetido a duas baterias de exames laboratoriais e de imagem em um período de três meses. Posteriormente, foi iniciado o tratamento com pimobendan e suplementação de taurina, resultando em resposta positiva e melhora dos sinais clínicos do paciente. Os achados ecocardiográficos do caso foram compatíveis com cardiomiopatia dilatada com sinais evidentes de diminuição progressiva das frações de ejeção, bem como encurtamento e aumento expressivo das câmaras cardíacas, quando se comparou este caso ao de cães de grande porte e animais saudáveis da mesma espécie. O tratamento com inotrópico positivo, suplementação dietética de taurina e diuréticos se mostrou eficiente em controlar os sinais clínicos do animal.(AU)

The dilated cardiomyopathy it is a chronic disease that leads to a cardiac dysfunction, resulting in unstable blood circulation and specimen body homeostasis. This description shows the dilated cardiomyopathy evolution and treatment in a giant anteater captive model. The patient presented cardiac insufficient clinical condition and was submitted to two sets of laboratorial and image exams in three months. Furthermore, the treatment started with pimobendam and taurine supplementation, leading to satisfactory response to treatment and clinical improvement. The echocardiographic findings were compatible with dilated cardiomyopathy, moreover clear evidence of progressive reduction at the ejection portions and shortening and expressive increase of the cardiac chamber when compared to large dogs and healthy animals of the same species. Treatment with positive inotropic and taurine dietary supplement revealed as effective in clinical managementr.(AU)

Severe reversible hypocalcemic cardiomyopathy diagnosed 36 years after subtotal thyroidectomy - a case report.

Chronic hypocalcemia, irrespectively of its etiology, can lead to severe impairment of the left ventricular (LV) contractility manifesting as dilated cardiomyopathy, usually defined as hypocalcemic cardiomyopathy (hypocaCM). This rarely diagnosed type of heart failure (HF), can be completely reversible, when treated properly with calcium and vitamin D supplementation or, in some subjects, with human recombinant parathormone. A CASE REPORT: The authors present a case of a 60-year-old male admitted with advanced pulmonary congestion, recurrent pulmonary edema and pleural effusion. He was refractory to standard HF therapy. One year earlier, the patient was diagnosed with primary dilated cardiomyopathy with ejection fraction decreased to 25% and after coronary angiography, which revealed normal coronary arteries, he received implantable cardioverter-defibrillator (ICD). However, 36 years before, the patient underwent subtotal thyroidectomy, and at the moment presented with multiplicitous symptoms suggestive of profound hypocalcemia. Laboratory investigations confirmed hypocalcemia due to iatrogenic hypoparathyroidism. Improvement of LV function during supplementation with calcium and vitamin D was monitored by echocardiography using two-dimensional longitudinal strain. One should always consider reversible causes of HF, including hypocalcemia, especially when obvious consequences of chronic hypocalcemia as myopathy, depression or cataract are seen. Correct therapy can restore LV function and prevent unnecessary implantation of ICD.

Dilated Cardiomyopathy as the Only Clinical Manifestation of Carnitine Transporter Deficiency.

The authors present a case of carnitine transporter deficiency, which was unmasked after an episode of respiratory distress resistant to treatment with bronchodilators. Chest radiograph showed cardiomegaly; electrocardiogram showed left ventricular hypertrophy and echocardiography revealed dilated cardiomyopathy. Heart failure therapy was initiated and metabolic screening was requested, as family history was indicative of inborn errors of metabolism. Very low levels of free carnitine and carnitine esters in blood were found and genetic testing confirmed the diagnosis of carnitine transporter deficiency. After oral supplementation with L-carnitine, symptoms gradually ameliorated and heart function had fully recovered. Sequence analysis in the SLC22A5 gene revealed the missense mutation c.1319C > T (p.Th440Met) in homozygous state. Homozygous c.1319C > T (p.Th440Met) mutation has not been associated with a pure cardiac phenotype before.

Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy.

BACKGROUND: Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined. OBJECTIVE: We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM. METHODS: A single-center retrospective review of children with DCM was performed. LTEs were defined as documented ventricular tachycardia or fibrillation requiring medical intervention. Three pediatric cardiologists, blinded to clinical events, evaluated ECGs obtained at the time of initial referral. Kaplan-Meier survival and Cox proportional hazards analyses were used to evaluate time to LTEs. RESULTS: A total of 137 patients (mean age 7.8 ± 6.7 years; 75(55%) male patients) with DCM (mean ejection fraction 35% ± 16%) were included; 67 patients (49%) had a corrected JT (JTc) interval of ≥340 ms, 72 (53%) had a corrected QT (QTc) interval of ≥450 ms, and 41 (30%) had abnormal T waves. LTEs occurred in 15 patients at a median of 12 months (interquartile range 3-36 months) after the initial ECG. Patients with LTEs had a longer JTc interval (371 ± 77 ms vs 342 ± 41 ms; P = .02) and a longer QTc interval (488 ± 96 ms vs 453 ± 44 ms; P = .01). In survival analysis, a JTc interval of ≥390 ms (hazard ratio [HR] 4.07; 95% confidence interval [CI] 1.12-14.83; P = .03), a QTc interval of ≥510 ms (HR 6.95; 95% CI 1.53-31.49; P = .01), abnormal T-wave inversion (HR 11.62; 95% CI 2.75-49.00; P = .001), and ST-segment depression (HR 6.91; 95% CI 1.25-38.27; P = .03) were associated with an increased risk of LTEs, even after adjusting for QRS duration and amiodarone use. CONCLUSION: Repolarization abnormalities are common in children with DCM. Certain ECG repolarization abnormalities, such as significantly prolonged JTc and QTc intervals, may be useful in identifying patients at risk of LTEs.

Cardiomyopathy responsive to gluten withdrawal in a patient with coeliac disease.

A 57-year-old man with iron deficiency anaemia developed general malaise, exertional dyspnoea and features of cardiac failure out of proportion to his anaemia (haemoglobin 120 g/L). Investigations showed a severely dilated left ventricle with an ejection fraction of 15%, due to dilated cardiomyopathy. He was treated with high-dose diuretics, ACE inhibitors and ß-blocker therapy. Subsequent investigation into his iron deficiency anaemia revealed a new diagnosis of coeliac disease. After starting a gluten-free diet, his cardiac function improved markedly, with ejection fraction reaching 70%, allowing his cardiac medications to be withdrawn. This case suggests a link between coeliac disease and cardiomyopathy.