scholarly journals Pathophysiological Complexity of Amiodarone-Induced Hypothyroidism (AIH) in patient With Congenital Heart Disease- Ebstein Anomaly: a case report

2021 ◽  
Author(s):  
Zvonimir Bosnic ◽  
Domagoj Vucic ◽  
Nikica Marinic ◽  
Blazenka Saric ◽  
Ljiljana Trtica Majnaric
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Systematic Study ◽  
Older Patients ◽  
Congenital Heart ◽  
Amiodarone Therapy ◽  
Class Iii ◽  
Ebstein Anomaly ◽  
Autoimmune Thyroid ◽  
Life Threatening

Abstract Background: Amiodarone is a class III antiarrhythmic drug, used for the threatment of life threatening supraventricular and ventricular tachyarrhythmias and widely used in prevention of life threatening tachyarrhythmia in adults with congenital heart disease. Thyroid dysfunction is a potentially serious complication of amiodarone therapy, especially in older patients with acquired heart disease. Cause there is no published systematic study on amiodarone-associated hypothirodism in patients with congenital heart disease, we report the case of a patient with Ebstein anomaly, a rare congenital heart disorder, who developed hypothyroidism after prolonged amiodrone therapy. We report a possible pathophysiological link of amiodarone-induced hypothyroidism in a patient with rare congenital heart disease, that has not been reported in a significant number in the literature so far.Case presentation: A female, 55-year-old patient with history of Ebstein anomaly (non operated), presented to a family medicine doctor with symptoms of progressive dyspnoea, fatigue and dysfagia for several months. Electrocardiography, echocardiography and chest X- ray confirmed presence of Ebstain anomaly. Laboratory results (including thyroid hormone values) and thyroid ultrasound were performed which confirmed the presence of hypothyroidism.Conclusions: According to available data, there is no published systematic study on amiodarone-associated hypothirodism in patients with congenital heart disease (large cohort studies). Pathophysiological complexity, however, could be due to the agent triggering autoimmune thyroid disease, so it is possible that amiodarone precipitated the onset of preexisting autoimmune disease. The value of regular thyroid function testing and measurement of thyroid antibodies should be considered in patients during amiodarone administration, especially in older patients with rare congenital heart disease.

scholarly journals Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension

Heart ◽  
2018 ◽  
Vol 104 (14) ◽  
pp. 1195-1199 ◽  
Author(s):  
Nika Skoro-Sajer ◽  
Christian Gerges ◽  
Olga Hajnalka Balint ◽  
Dora Kohalmi ◽  
Monika Kaldararova ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Mixed Model ◽  
Adult Patients ◽  
Class Iii ◽  
Infusion Site ◽  
Pulmonary Arterial

ObjectiveTo assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment.MethodsConsecutive adult patients with CHD–PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study.ResultsAdvanced CHD–PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13). In the multivariate mixed model, mean increase in 6 min walk distance (6-MWD) from baseline to 12 months was 114 m (76; 152) (P<0.001). WHO FC improved significantly (P=0.001) and B-type brain natriuretic peptide decreased from 1259 (375; 2368) pg/mL to 380 (144; 1468) pg/mL (P=0.02). In those 14 patients who had haemodynamic data before and after initiation of treprostinil, pulmonary vascular resistance decreased significantly (from 18.4±11.1 to 12.6±7.9 Wood units, P=0.003). The most common adverse events were infusion-site erythema and pain. One patient stopped treatment because of intolerable infusion-site pain after 8 months of treatment. No other major treatment-related complications were observed. Five patients died during early follow-up, having experienced a decrease in their 6-MWD prior.ConclusionsSubcutaneous treprostinil therapy is generally safe and effective for at least 12 months and may be used in CHD-related PAH class III and IV.

scholarly journals Coxiella Endocarditis as the Cause of Recurrent Fever and Brain Abscess in a Patient with Complex Congenital Heart Disease: A Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Ioannis A. Ziogas ◽  
Alexandros P. Evangeliou ◽  
Olga Tsachouridou ◽  
Alexandra Arvanitaki ◽  
Afroditi Tsona ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Case Report ◽  
Congenital Heart ◽  
Diagnostic Delay ◽  
Unknown Origin ◽  
Recurrent Fever ◽  
Complex Congenital Heart Disease ◽  
Empiric Antibiotic ◽  
Life Threatening

Introduction. Blood culture-negative infective endocarditis (BCNIE) can present subtly and is associated with a diagnostic delay leading to increased morbidity and mortality. Case Report. We present the case of an 18-year-old male with a history of complex congenital heart disease and 3-year intermittent episodes of fever of unknown origin, who was referred to our hospital for upper and lower extremity focal seizures. Laboratory blood tests were normal, blood cultures were negative, and brain imaging revealed an abscess. Cardiology consultation was requested, and transthoracic echocardiography revealed an intracardiac vegetation. Empiric antibiotic treatment with sultamicillin, gentamycin, and meropenem was initiated. Serology testing was positive for Coxiella burnetii, and the diagnosis of BCNIE was established. The antibiotic course was changed to oral doxycycline for 36 months and led to resolution of IE, with no vegetation detected on TTE after 15 months. Conclusion. BCNIE is a life-threatening disease entity that can lead to severe complications, such as valve regurgitation, emboli, and death. Patients with congenital heart disease are particularly vulnerable to IE. Timely diagnosis and antibiotic management are of paramount importance in order to avoid the potentially fatal sequelae.

scholarly journals P879 3D transoesophageal echocardiography to asses a case of a non-ebstein tricuspid valve congenital regurgitation

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Sanz Ortega ◽  
S Velasco Del Castillo ◽  
J J Onaindia Gandarias ◽  
I Rodriguez Sanchez ◽  
J Florido Perena ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Congenital Heart ◽  
Volume Overload ◽  
Transoesophageal Echocardiography ◽  
Ebstein Anomaly ◽  
Downward Displacement ◽  
And Function

Abstract Introduction Due to the complexity of congenital heart disease and limitations of transthorathic echocardiogram (TTE), especially in adult patients, it is not unusual to need other image techniques to assess cardiac anatomy and function. The most common primary anomaly of tricuspid valve (TV) is Ebstein anomaly, but there are other much rarer primary anomalies of this valve consisting in prolapse, cord retraction.... without downward displacement of the leaflet, generally causing tricuspid regurgitation (TR) that can be severe and sometimes intervention is needed, preferably reparation. Due to anatomical issues, it is difficult to assess anatomy of TV in TTE, so sometimes 3D-TTE must be performed to clarify the mechanism and to measure orifice, but when transthoracic view is not enough, 3D transoesophageal echocardiogram (TOE) can be useful for this purpose. Case We report the case of a 15-year-old boy that was referred to our clinic because of shortness of breath and a systolic tricuspid murmur. TTE was performed and an image compatible with tricuspid valve prolapse with no apical displacement of any leaflets (Figure, A) causing severe TR (Figure, B) was noticed, as well as severely dilated right chambers, with good ejection fraction of both ventricles. It was not clear the mechanism so 2D TOE was done, showing a prolapse of a leaflet (Figure, C) causing severe TR (Figure, D). The mechanism was finally clarified by 3D TOE (figure E). This was a prolapse of lateral portion of posterior leaflet (asterisk) with restrictive movement of anterior (triangle) and septal (arrow) ones, causing a huge coaptation defect in systole leading to a very severe tricuspid insufficiency with signs of volume overload of right ventricle. There was no atrial septal defect and pulmonary drainage anomalies were ruled out by cardiac magnetic resonance. Patient was referred to surgery due to symptoms and great dilatation of right chambers. Conclusión: Due to anatomical complexity and limitations of echography, cross and multimodality cardiac imaging is usually needed in assessing congenital heart disease. Apart from Ebstein anomaly, other congenital entities of tricuspid valve such as prolapse and/or retraction can lead to severe tricuspid regurgitation. Due to limitations of 2D TTE in assessing tricuspid valve anatomy, 3D TTE has to be performed, but if it is not enough, 3D TOE can be an option to evaluate mechanism and directly see the orifice of regurgitation in congenital disease of tricuspid valve. Abstract P879 Figure

Reconstruction of a Previously Repaired Aortic Valve Destroyed by Infective Endocarditis: Case Report

2016 ◽  
Vol 8 (3) ◽  
pp. 408-410
Author(s):  
Tomas Chalela ◽  
Viktor Hraska
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Case Report ◽  
Infective Endocarditis ◽  
Aortic Valve ◽  
Congenital Heart ◽  
Congenital Aortic Stenosis ◽  
Life Threatening ◽  
History Of ◽  
Uncommon Condition

Infective endocarditis (IE) is an uncommon condition among patients with congenital heart disease, however it can be life threatening. The usual management includes replacement of the affected valve, especially in patients with aortic valve compromise, and is even more common in previously repaired valves. In this case report, we describe the successful reconstruction of an aortic root destroyed by IE, in a patient with history of ballooning of a congenital aortic stenosis.

scholarly journals Incidence and Management of Life-Threatening Adverse Events During Cardiac Catheterization for Congenital Heart Disease

2013 ◽  
Vol 35 (1) ◽  
pp. 140-148 ◽  
Author(s):  
C. Huie Lin ◽  
Sanjeet Hegde ◽  
Audrey C. Marshall ◽  
Diego Porras ◽  
Kimberlee Gauvreau ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adverse Events ◽  
Cardiac Catheterization ◽  
Congenital Heart ◽  
Life Threatening

scholarly journals Long-term outcomes after myocardial infarction in middle-aged and older patients with congenital heart disease—a nationwide study

2020 ◽  
Author(s):  
Maria Fedchenko ◽  
Zacharias Mandalenakis ◽  
Kok Wai Giang ◽  
Annika Rosengren ◽  
Peter Eriksson ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Older Patients ◽  
Congenital Heart ◽  
Long Term Outcomes ◽  
Increased Risk ◽  
New Onset

Abstract Aims  We aimed to describe the risk of myocardial infarction (MI) in middle-aged and older patients with congenital heart disease (ACHD) and to evaluate the long-term outcomes after index MI in patients with ACHD compared with controls. Methods and results  A search of the Swedish National Patient Register identified 17 189 patients with ACHD (52.2% male) and 180 131 age- and sex-matched controls randomly selected from the general population who were born from 1930 to 1970 and were alive at 40 years of age; all followed up until December 2017 (mean follow-up 23.2 ± 11.0 years). Patients with ACHD had a 1.6-fold higher risk of MI compared with controls [hazard ratio (HR) 1.6, 95% confidence interval (CI) 1.5–1.7, P &lt; 0.001] and the cumulative incidence of MI by 65 years of age was 7.4% in patients with ACHD vs. 4.4% in controls. Patients with ACHD had a 1.4-fold increased risk of experiencing a composite event after the index MI compared with controls (HR 1.4, 95% CI 1.3–1.6, P &lt; 0.001), driven largely by the occurrence of new-onset heart failure in 42.2% (n = 537) of patients with ACHD vs. 29.5% (n = 2526) of controls. Conclusion  Patients with ACHD had an increased risk of developing MI and of recurrent MI, new-onset heart failure, or death after the index MI, compared with controls, mainly because of a higher incidence of newly diagnosed heart failure in patients with ACHD. Recognizing and managing the modifiable cardiovascular risk factors should be of importance to reduce morbidity and mortality in patients with ACHD.

Adverse Effects of Amiodarone Therapy in Adults With Congenital Heart Disease

2018 ◽  
Vol 27 ◽  
pp. S391 ◽  
Author(s):  
B. Moore ◽  
R. Cordina ◽  
M. McGuire ◽  
D. Celermajer
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adverse Effects ◽  
Congenital Heart ◽  
Amiodarone Therapy
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