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  • Author: Lina Gumbienė x
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Pregnancy in long QT syndrome with implantable cardioverter-defibrillator. A case report and review of the literature

Summary

Implantable cardioverters-defibrillators have decreasedmorbidity and mortality as well as improved quality of life in patients with life-threatening cardiac arrhythmias and allowed an increasing number of young women to reach their reproductive years. New questions and tasks arise for medical professionals as to organize appropriate management of these patients, because little is known regarding the risk and outcomes of such pregnancies. The aim of this report is to describe our centre’s first experience of pregnancy and delivery management in patient with an implantable cardioverter-defibrillator as primary prevention of ventricular arrhythmias in congenital long QT syndrome.

Open access
Case report: Life-saving pulmonary endarterectomy for a young female with primary antiphospholipid syndrome complicated by severe chronic thromboembolic pulmonary hypertension

Abstract

Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary antiphospholipid syndrome. Pulmonary endarterectomy - the surgical removal of the organized thromboembolic material from the proximal pulmonary arteries - is the procedure of choice and potentially a curative option for patients with chronic thromboembolic pulmonary hypertension, including patients with antiphospholipid syndrome. We report an exceptionally severe and complicated case with favourable outcome. Case presentation: We present a case of a successful high risk pulmonary endarterectomy in a 29-year-old female with primary antiphospholipid syndrome and end-stage chronic thromboembolic pulmonary hypertension. Despite highly complicated perioperative course an impressive improvement in symptoms and functional status was achieved. Conclusion: We hope that this complicated but successfully managed case of a combination of two rare diseases will arouse earlier suspicion and timely diagnosis for such patients and will encourage physicians to promptly refer the suitable patients to a pulmonary endarterectomy team. It is important to remember that severe thrombocytopenia may occur in patients with antiphospholipid syndrome

Open access
Late arrhythmia in patients with repaired tetralogy of Fallot: who is at risk?

Summary

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart defect. Over the last century, the life expectancy of TOF patients has significantly improved. This, however, has brought new challenges both to patients and their health-care providers, the main of them being late arrhythmia. Ironically, late arrhythmia is predominantly generated due to the fibrotic scars caused by the life-saving surgical repair. Once the first two mainly arrhythmia-free decades after the repair pass, the risk of developing late arrhythmia and, therefore, SCD becomes substantial. Consequently, young adults with repaired Tetralogy of Fallot (rTOF) require careful outpatient monitoring.

There have been many attempts to predict the risk of life-threatening arrhythmia in rTOF patients. This has led to the defining of various risk factors, ranging from the widely used QRS prolongation to novel predictors, derived from cardiac magnetic resonance (CMR) based anatomical findings (left ventricular dyssynchrony indexes, right ventricular output tract akinetic length, right ventricular mass-to-volume ratio). The latter predictors have recently established CMR as a tool of high significance in evaluation of rTOF patients.

Although the role of Holter monitoring findings in rTOF patient-assessment remains unclear, it may be useful in those who are 25 years and older. Implantable cardioverter–defibrillator (ICD) implantation is the first-line treatment for secondary prevention of sudden cardiac death (SCD). rTOF patients suffer from the highest rate of inappropriate and the lowest rate of appropriate ICD shocks, when compared to other congenital heart diseases. As a consequence, ICD implantation for primary SCD prevention should be carefully weighed. Catheter-based ablation therapy leads to high rate of initial success when abolishing monomorphic ventricular tachycardia and intra-atrial reentrant tachycardia, however recurrence rates remain high.

Open access
Multidetector computed tomographic angiography in evaluation of neonates with suspected extracardiac congenital heart disease: experience in Vilnius University Hospital Santariškių Klinikos

Summary

The aim of this study was to determine the frequency of extracardiac pathology in neonatal patients with suspected congenital heart disease referred to undergo multidetector computed tomography (MDCT) angiography in Vilnius University Hospital Santariskiu Klinikos. The safety of MDCT angiography (intravenous contrast media volume injected, scanning time and exposure to ionising radiation) was also evaluated.

Patients and Methods: During the period of 2006-2012, in Vilnius University Hospital Santariskiu Klinikos 186 pediatric patients were referred to undergo MDCT angiography of whom 71 were neonates with suspected complex congenital heart disease (CHD) and extracardiac pathology.

Results: Sixty nine (97.2%) of referred neonates were found to have extracardiac pathology (36 had extracardiac vascular pathology only, 4 - extracardiac non-vascular pathology only and 29 - both extracardiac vascular and non-vascular pathology). In 47 patients diagnosis of extracardiac pathology was verified intraoperatively. Remainder (n = 24) of the group were not operated (12 patients died because of hemodynamic instability and inoperable complex CHD, 9 patients were scheduled for later surgery, in 3 patients surgery was not indicated). Mean contrast media volume used was 4.18±1.03 ml, mean scanning time was 1.07±0.77 s and mean effective radiation dose was 2.8± 0.69 mSv. No adverse reactions or complications were observed.

Conclusions: MDCT angiography is accurate and safe method in diagnosis of complex congenital heart disease with extracardiac pathology.

Open access