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  • Open Access Icon
  • Research Article
  • 10.20418/jrcd.v0i0.409.g327
Association of fibrinogen and D‑dimer levels with severity of acute coronary syndromes
  • Jan 1, 2020
  • Journal of Rare Cardiovascular Diseases
  • Mukhyaprana M Prabhu + 3 more

Background: Acute coronary syndromes (ACSs) are the primary cause of mortality worldwide. The aim of the study was to assess the as‑sociations of serum fibrinogen and plasma D‑dimer levels with angiographic severity of atherosclerotic lesions as well as the presence of in‑hospital complications and complications at 30‑day follow‑up in patients with ACS. Methods: This was a prospective study including 107 patients with ACS. Severity of CAD was assessed by the Gensini score. Correlations of D‑dimer and fibrinogen levels with complica‑tions such as heart failure, arrhythmia, recurrent angina, and cardiac death were assessed using the Pearson correlation coefficient and the receiver operating characteristic curve analysis. Results: The mean age of patients was 61±10.9 years. Mean serum fibrinogen levels were higher in individuals with severe left ventricular (LV) dysfunction than in those with moderate and mild LV dysfunction (444 mg/dl, 404 mg/dl, and 330 mg/dl, respectively). Similarly, the mean plasma D‑dimer level was higher in individuals with severe ACS (1.03 μg/ml) than in those with moderate (1.88 μg/ml) and mild ACS (3.5 μg/ml). Conclusion: Our study revealed that patients with higher serum fibrinogen levels tend to have more severe ACS, greater LV dysfunction, and a higher rate of complications. Therapies aimed at reducing fibrinogen levels might help reduce mortality and morbidity in patients with ACS.

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  • Research Article
  • 10.20418/jrcd.vol4no3.384
Prevalence and characteristics of patients with solitary coronary artery fistulas in 12,757 all comer adult patients undergoing coronary angiography (RCD code: I 1C.4)
  • Jan 1, 2020
  • Journal of Rare Cardiovascular Diseases
  • Jakub Chmiel + 7 more

Introduction Coronary artery fistula (CAF) is a rare anomaly, mostly congenital, defined as an abnormal direct connection between one of the coronary arteries and a heart chamber or major vessel. In case of coronary steal, CAF may manifest with symptoms of stable coro‐ nary disease (CAD). Methods We investigated medical records of all adult patients who underwent coronary angiography (CAG) from 1 July 2009 to 30 June 2019. Patients with solitary CAF were selected. Coronary artery‐ventricular multiple microfistulas (MMFs) were recorded but were no object for further analysis. The CAFs were grouped depending on the origin and termination. Clinical symptoms, comorbidities and past medical history were analyzed. Results 26 solitary CAFs were found in 22 (0.17%) and 20 MMFs in 16 (0.13%) out of 12,757 patients who underwent CAG for any reason in that period. Indication for CAG varied among study group. Left coronary artery (LCA) gave origin to majority (57.69%) of fistulas. Pulmonary artery (PA) was the most common drainage site (69.23%). 17 patients had HA, 15 suffered from chest pain or dyspnoea, 13 had dyslipidemia, and half had CAD. Conclusion The incidence of solitary CAF in the sample of all‐comer population of Polish patients undergoing CAG was 0.17%. LCA was the most common artery of origin and PA was the most frequent drainage site. The indication for CAG varied among patients. Majority of patients suffered from chest pain or dyspnea, had HA, dyslipidemia, and half had CAD. Moreover, the incidence of MMF was 0.13%. JRCD 2019; 4 (3): 42-46.

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  • Research Article
  • 10.20418/jrcd.vol4no3.357
Critical value of the balloon occlusion test of a coronary fistula in a patient with pulmonary atresia and intact ventricular septum (RCD code: I 1C.4;­ II 2A.1)
  • Jan 1, 2020
  • Journal of Rare Cardiovascular Diseases
  • Jacek Kuźma + 4 more

We present the case of a boy with pulmonary atresia (PA), intact ventricular septum (IVS), and a coronary fistula between the right ventricle (RV) and a single left coronary artery (SLCA). In the newborn period, the child was operated on using a right Blalock–Taussing shunt (RBTS). At the age of 6 months, he was admitted to the department of Paediatric Cardiology due to hypoxaemia and cardiac catheterisation was performed before qualification for cardiac surgery. During haemodynamic evaluation of the patient, we conducted the balloon occlusion test of the coronary fistula and demonstrated that coronary circulation depends on the wide fistula and high pres‐ sure in the RV. We decided not to perform embolisation of the fistula and qualified the child for bidirectional Glenn palliation without RV decompression. The balloon occlusion test in patients with coronary fistulas plays a critical role and its result can be crucial for further management of the patient. JRCD 2019; 4 (3): 55-58.

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  • Research Article
  • 10.20418/jrcd.vol4no3.387
Tachycardia induced cardiomyopathy due to dual atrioventricular nodal non reentrant tachycardia (RCDD code: VI 1A 9)
  • Jan 1, 2020
  • Journal of Rare Cardiovascular Diseases
  • Ulyana Chernyaha-Royko + 5 more

Dual atrioventricular nodal non‐reentrant tachycardia (DAVNNT) is a rarely diagnosed, sustained supraventricular arrhythmia. We report the case of a 39‐year‐old female with drug‐resistant, irregular, narrow QRS complex tachycardia which gradually led to tachycardia‐induced cardiomyopathy. Previously, this arrhythmia was recognized as atrial fibrillation, with several unsuccessful attempts at pulmonary vein isolation. Only after the correct diagnosis of DAVNNT was established, successful radiofrequency ablation of the slow pathway in the atrioventricular node was performed, with complete cure of the arrhythmia. JRCD 2019; 4 (3): 59-62.

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  • Research Article
  • 10.20418/jrcd.vol4no1.346
Hypertrophic cardiomyopathy – symptomatic atrial fibrillation in a patient at high risk of sudden cardiac death (RCD code: V-2A.2)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Adam Gębka + 3 more

We present the case of a young patient with significant left ventricular hypertrophy as a common representation of the hypertrophic cardiomyopathy (HCM) phenotype. The clinical presentation and diagnostic route of the disease (despite negative genotype), which can be found in everyday cardiology practice, are shown. Despite the presence of guidelines on this topic, each clinical case is demanding, especially during qualification for invasive procedures. Limited data about the periprocedural risk of catheter ablation and success rate in HCM makes physician decisions for this type of patient challenging. The importance of informed consent and how the patient’s decisions affet further progress are also shown. JRCD 2018; 4 (1): 18-21.

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  • Research Article
  • 10.20418/jrcd.vol3no8.348
Non‐ruptured symptomatic splenic artery aneurysm (RCD code: I-1D.1)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Hossein Farsavian + 7 more

Splenic artery aneurysm occurs in 1% of the population. Most splenic artery aneurysms are asymptomatic and are diagnosed incidentally Symptomatic splenic artery aneurysm is usually detected due to rupture, while non‐ruptured splenic artery aneurysm is rare We present the case of a 69‐year‐old female who presented with signs of left abdominal pain and vomiting, and was diagnosed with splenic artery aneurysm. Diagnosis was made by CT scan and revealed a non‐ruptured splenic artery aneurysm. Open abdominal surgery, endovas‐ cular treatment and laparoscopic surgery are treatment options for splenic artery aneurysms. Immediate treatment after diagnosis of symptomatic splenic artery aneurysm is recommended. JRCD 2018; 3 (8): 275–277

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  • Research Article
  • 10.20418/jrcd.vol4no1.341
Primary hyperaldosteronism presenting as recurrent polymorphic ventricular tachycardia (RCDD code: VI-2)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Marian Hrebenyk + 2 more

Primary hyperaldosteronism (PH) is a frequently overlooked cause of secondary hypertension mostly due to an aldosterone-producing adrenal adenoma. We report the case of a 48-year-old female who presented with recurrent episodes of polymorphic ventricular tachycardia before an aldosterone-producing adrenal adenoma was diagnosed. Although her past medical history was remarkable for poorly-controlled hypertension since age 30, she had never been examined for secondary causes. The patient underwent adrenalectomy resulting in stabilisation of her clinical condition, although residual hypertension remained. Causes of atypical presentation and diagnostic pitfalls are discussed. JRCD 2018; 4 (1): 26-30.

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  • Research Article
  • 10.20418/jrcd.vol4no2.361
Foetal 2:1 atrioventricular block in a patient with Timothy syndrome (LQT8) (RCDD code: VI-1B-1.2)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Putri Yubbu + 4 more

Long QT syndrome (LQTS) may be a cause of foetal bradyarrhythmia and an important cause of death in children with arrhythmia. We present the case of a patient of Kadazan Iban descent with LQTS. He was detected prenatally to have foetal 2:1 atrioventricular (AV) block and tetralogy of Fallot. His postnatal electrocardiogram revealed a functional 2:1 AV block with QTc interval of 690 ms. Dysmorphism and cutaneous syndactyly of both hands and feet pointed to a diagnosis of classical Timothy syndrome (TS) type 1. This diagnosis was confirmed molecularly with a heterozygous mutation c.1216G>A. p. (Gly406Arg) at exon 8A in the CACNA1C gene. To the best of our knowledge, this is the first reported case of TS in a Kadazan Iban child. JRCD 2019; 4 (2): 42-46.

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  • Research Article
  • 10.20418/jrcd.vol4no1.349
Superior vena cava syndrome associated with implantable cardiac devices procedures (RCD code: VIII)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Szymon Jonik + 1 more

INTRODUCTION: Superior vena cava (SVC) syndrome is a rare clinical disorder associated with obstructing venous outflow through the SVC. Although the most common cause is mediastinal tumors, the growing number of implantable cardiac devices procedures has resulted in more patients with SVC syndrome of non‐malignant etiology. DISCUSSION: It has been shown repeatedly that the use of cardiac implantable electronic devices (CIEDs) improves the quality of life of patients with symptomatic arrhythmias and reduces the mortality of patients at risk of sudden cardiac death, but the risk of complications also exists. CONCLUSION: The most common finding in patients with cardiac devices is asymptomatic venous thrombosis, but serious complications, including SVC obstruction should always be considered. The rapid identification of clinical symptoms and the implementation of appropriate measures are a key to preventing the morbidity and mortality of the patients. JRCD 2018; 4 (1): 5-8.

  • Open Access Icon
  • Research Article
  • 10.20418/jrcd.vol4no2.363
Current views on the use of interferons in the treatment of polycythaemia vera (RCD code: VIII)
  • Jan 1, 2019
  • Journal of Rare Cardiovascular Diseases
  • Anna Prochwicz + 2 more

Interferon alpha is a molecule associated with stimulation of immune system cells, resulting in an anti‐proliferative and immunomodulatory effect. It has been demonstrated that interferon reduces the number of platelets, leukocytes, and erythrocytes in patients suffering from chronic myeloproliferative diseases. In this paper, we present an overview of selected research evaluating the efficacy and adverse effects of various recombinant interferons used in the treatment of polycythaemia vera. We have analysed previously reported studies on the use of interferon. Interferon alfa‐2a was the first interferon approved for standard treatment of polycythaemia vera, while the next was pegylated interferon alfa‐2a. We also present recent results from studies on a newly modified molecule, ropeginterferon, a mono‐pegylated form of interferon alfa‐2b. Interferons reduce the number of phlebotomies required in patients with polycythaemia vera, accompanied by a resolution of typical disease symptoms. Treatment is well tolerated by the majority of patients. JRCD 2019; 4 (2): 34-36.