Presentation
The exclusion of a large akinetic RVOT area during reoperation of patients presenting with severe post-repair right ventricular dilatation was investigated. [ncbi.nlm.nih.gov]
Pediatric patients usually have no symptoms but patients over 40 years of age are symptomatic at presentation [ 5 ]. [omicsonline.org]
Because of the progressive nature of the disorder, patients may present with any number of symptoms. [af-ablation.org]
Case Report Case presentation This is a 28-year-old male who presented to the emergency department for traveler’s diarrhea that resolved the following day. [jxym.amegroups.com]
Entire Body System
- Asymptomatic
A case of asymptomatic patient with right ventricular dilatation. J Xiangya Med 2016;1:9. [jxym.amegroups.com]
BACKGROUND: Cardiac volumetry by magnetic resonance imaging can guide the timing for reoperation in minimally symptomatic or asymptomatic patients with pulmonary insufficiency after corrected tetralogy of Fallot. [ncbi.nlm.nih.gov]
Thus in the absence of atrial septal defect this anomaly should be considered in case of right heart dilatation in asymptomatic children. The vast majority of such anomalies were treated surgically in the past. [omicsonline.org]
Asymptomatic patients usually do not require further treatment. Symptomatic patients Beta blockers ; ( 1 st line ) or verapamil ( 2 nd line ). [amboss.com]
It is important to be able to recognize these ECG changes because hypertrophy is associated with adverse cardiovascular outcome (increased risk of heart failure and arrhythmia), even though the patient may be asymptomatic for several years. [ecgwaves.com]
Gastrointestinal
- Regurgitation
Even after adjusting for pulmonary regurgitation, tricuspid regurgitation was significantly correlated with right ventricular volume (r= 0.39, p = 0.009), suggesting that tricuspid regurgitation as well as pulmonary regurgitation may contribute significantly [ncbi.nlm.nih.gov]
Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur Heart J. 2009;30(3):356–61. [jcmr-online.biomedcentral.com]
Concomitant tricuspid annuloplasty was performed in 13 patients with grade 3 or 4 tricuspid regurgitation. In 2004, 102 patients underwent mitral valve repair. [hartlongcentrum.nl]
- Diarrhea
Case Report Case presentation This is a 28-year-old male who presented to the emergency department for traveler’s diarrhea that resolved the following day. [jxym.amegroups.com]
Diarrhea announces the congested condition of the intestinal tract, and adds to the debility. The kidneys are also frequently involved, and nephritis is a common attendant, the urine being scanty, high-colored, albuminous, and contains casts. [henriettes-herb.com]
Cardiovascular
- Heart Disease
Infants, children and adolescents with congenital heart disease (CHD) are a challenge to manage and an ever-increasing number are reaching adulthood. [books.google.de]
ICD-10-CM Codes › I00-I99 Diseases of the circulatory system › I30-I52 Other forms of heart disease › I51- Complications and ill-defined descriptions of heart disease › Cardiomegaly 2016 2017 2018 2019 Billable/Specific Code I51.7 is a billable/specific [icd10data.com]
[…] artery disease or heart attack cause the disease. [heart.org]
Idiopathic myocarditis associated with such unusual features is relatively rare, and may present problems in differentiation from other congenital heart diseases causing dilatation and dysfunction of the right ventricle. [ncbi.nlm.nih.gov]
- Cyanosis
There was no evidence of anemia, jaundice, cyanosis, or clubbing. The respiratory system was within normal limits. Cardio-vascular system examination showed normal S1 and S2 with no added sounds. [ijpmonline.org]
[…] finding on exam was tachypnea (60%).(2) Several abnormalities on the physical exam which suggest RV dysfunction include systemic arterial hypotension, right sided S3, accentuated P2, parasternal lift, tricuspid regurgitation murmur, elevation of JVP and cyanosis [rwjms1.umdnj.edu]
Cyanosis may be observed in patients with right to left shunting or severe low cardiac output. Echocardiography plays a key role in the diagnosis of right heart disease. [revespcardiol.org]
Musculoskeletal
- Myopathy
Like dilated myopathy, restrictive cardiomyopathy produces left and right heart failure. Ejection fraction is usually normal, but diastolic filling is reduced on echocardiography. [amboss.com]
Hydroxychloroquine can cause skeletal and cardiac myopathies. [clevelandclinicmeded.com]
Neurologic
- Akinesia
[…] used qualitative standards for diagnosing RV dysfunction are RV/LV end diastolic diameter >1 in apical 4 chamber view, RV end diastolic diameter >30 mm and paradoxical RV septal motion.(19) A distinct echocardiographic pattern of RV dysfunction, with akinesia [rwjms1.umdnj.edu]
Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. [jcmr-online.biomedcentral.com]
- Ataxia
[…] causes disorganized myocyte architecture characterized by myofibrillar disarray and fibrosis Autosomal dominant inheritance with varying penetrance ( familial occurrence in > 50% of cases ) Secondary hypertrophic cardiomyopathy ; (e.g., in Friedreich ataxia [amboss.com]
Cardiac MRI (CMRI) typically shows focal inferolateral midwall LGE sparing the subendocardium. [53, 54] Other infiltrative diseases Other infiltrative diseases that resemble hypertrophic/hypertensive heart disease include Danton disease, Friedreich ataxia [emedicine.medscape.com]
Workup
X-Ray
- Pericardial Effusion
Chest X-rays showed cardiac dilatation and hydrothorax; whilst there was no abnormal hyperkinesis on the echocardiogram, pericardial effusion was identified; diuretics and hANP administration was started. [kochi-med.or.jp]
manifested by pericardial effusion. [46] It is diagnosed via EMB or with noninvasive modalities, such as the following: Echocardiography: The echocardiographic appearance of cardiac amyloidosis includes LV and right ventricular (RV) wall thickness with [emedicine.medscape.com]
No pericardial effusion or mural thrombus was observed in any of the vessels or the heart chambers. [ijpmonline.org]
Serum
- Dyslipidemia
[…] catecholaminergic response, coronary vasospasm, and myocarditis. [23] HIV-associated cardiomyopathy Cardiac manifestations of infection with human immunodeficiency virus (HIV) include myocarditis, dilated cardiomyopathy, pericardial effusion, vasculitis, dyslipidemia [emedicine.medscape.com]
QRS Wave
- Poor R Wave Progression
Figure 3 shows atrial fibrillation, poor R wave progression, and diffuse ST-segment abnormalities in an ECG from a patient with ischemic cardiomyopathy. [clevelandclinicmeded.com]
Hypertrophy
- Right Ventricular Hypertrophy on ECG
[…] case due to right ventricular hypertrophy. [lifeinthefastlane.com]
Treatment
A triad of circulatory collapse, right ventricular dilatation, and large alveolar dead space is proposed for the rapid diagnosis and treatment of massive pulmonary embolism. [ncbi.nlm.nih.gov]
Treatment All patients should avoid strenuous exercise. Asymptomatic patients usually do not require further treatment. Symptomatic patients Beta blockers ; ( 1 st line ) or verapamil ( 2 nd line ). [amboss.com]
Standard treatment of right ventricular outflow tract or pulmonary artery obstruction involves open chest surgery. [nice.org.uk]
Treatments for dilated cardiomyopathy At present there is no cure for DCM, but treatments are available to help control your symptoms and prevent complications. Your treatment will depend on how your heart is affected and what symptoms you have. [bhf.org.uk]
Prognosis
Abstract The prognosis of multidetector computed tomography (MDCT) assessed right ventricular dilatation (RVD) is unclear in patients with pulmonary embolism (PE) and a simplified Pulmonary Embolism Severity Index (sPESI) of 0. [ncbi.nlm.nih.gov]
The prognosis of pulmonary hypertension (PH) depends upon the functional capacity of the right ventricle. [jscimedcentral.com]
The study of life prognosis in patients with 2 forms of DCM showed that 5-year mortality of patients was about 50%. [ijbm.org]
Our outcome figures suggest a guarded prognosis be given during counseling of parents of fetuses with right heart dilatation. [link.springer.com]
Etiology
Overview of major types of cardiomyopathy Dilated cardiomyopathy Epidemiology Most common cardiomyopathy Incidence : 6/100,000 per year Sex: ♂ > ♀ (approx. 3:1) Etiology Idiopathic (approx. 50%) Secondary cause Coronary heart disease Arterial hypertension [amboss.com]
[…] hypoplastic left heart syndrome interrupted aortic arch aortic coarctation ventricular septal defect 1 congenital pulmonary stenosis : tends to cause more wall hypertrophy rather than chamber dilatation dilated fetal cardiomyopathy more unusual cardiac etiologies [radiopaedia.org]
A systematic approach to define the etiology is essential for determination of the most effective treatment strategy. [emedicine.medscape.com]
Other studies should be individualized depending upon the suspected etiology of RHF. [revespcardiol.org]
Epidemiology
Overview of major types of cardiomyopathy Dilated cardiomyopathy Epidemiology Most common cardiomyopathy Incidence : 6/100,000 per year Sex: ♂ > ♀ (approx. 3:1) Etiology Idiopathic (approx. 50%) Secondary cause Coronary heart disease Arterial hypertension [amboss.com]
Epidemiology The incidence of acute cor pulmonale depends on the underlying cause. [clinicaladvisor.com]
Differences in left ventricular structure between black and white hypertensive adults: the Hypertension Genetic Epidemiology Network Study. Hypertens 2004; 43:1182–1188 View Article Google Scholar 18. [journals.plos.org]
[…] corticosteroids; thus, timely diagnosis via EMB is prudent. [13, 14] Treatment with cyclosporine and corticosteroids is associated with a median transplant-free survival of 12.3 months compared to 3 months without immunosuppression. [15] The European Study of Epidemiology [emedicine.medscape.com]
Pathophysiology
References: [2] [2] [7] [8] [9] [3] [10] [11] [12] [13] [14] [15] Restrictive cardiomyopathy Pathophysiology Proliferation of connective tissue → ↓ elasticity of myocardium → ↓ ventricular compliance → ↓ diastolic filling → atrial congestion → atrial [amboss.com]
“Acute right ventricular failure — from pathophysiology to new treatments”. Intensive Care Med. vol. 30. 2004. pp. 185 Thorough review of all aspects of acute cor pulmonale, with an emphasis on pathophysiology and diagnosis. [clinicaladvisor.com]
When a clot lodges in the pulmonary arterial circulation, various pathophysiologic effects occur. [rwjms1.umdnj.edu]
Right ventricular changes in highly trained athletes: Between physiology and pathophysiology. [jcecho.org]
Pathophysiology and management of right heart ischemia. J Am Coll Cardiol. 2002;40:(5)841-53. Isner JM, Roberts WC. Right ventricular infarction complicating left ventricular infarction secondary to coronary heart disease. [blog.5minsono.com]
Prevention
OBJECTIVES: We hypothesize that concomitant tricuspid annuloplasty in patients with tricuspid annular dilatation who undergo mitral valve repair could prevent progression of tricuspid regurgitation and right ventricular remodeling. [ncbi.nlm.nih.gov]
Objectives We hypothesize that concomitant tricuspid annuloplasty in patients with tricuspid annular dilatation who undergo mitral valve repair could prevent progression of tricuspid regurgitation and right ventricular remodeling. [hartlongcentrum.nl]
In some cases, this prevents the heart from filling with blood as it should. Over time, it can affect the other chambers. Symptoms Many people with dilated cardiomyopathy have no symptoms. Some that do have only minor ones, and live a normal life. [webmd.com]
His research aims to find better ways to diagnose, treat and prevent people dying from this disease, and beat the heartbreak. Donate [bhf.org.uk]