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Heart rate variability and ventricular late potentials after liver transplantation for familial amyloidotic polyneuropathy
Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
(English)Manuscript (Other academic)
Identifiers
URN: urn:nbn:se:umu:diva-2684OAI: oai:DiVA.org:umu-2684DiVA: diva2:140924
Available from: 2007-10-26 Created: 2007-10-26 Last updated: 2012-06-21Bibliographically approved
In thesis
1. Cardiac arrhythmias and heart rate variability in familial amyloidotic polyneuropathy: A clinical study before and after liver transplantation
Open this publication in new window or tab >>Cardiac arrhythmias and heart rate variability in familial amyloidotic polyneuropathy: A clinical study before and after liver transplantation
2007 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Familial amyloidotic polyneuropathy (FAP), found in the northernmost counties in Sweden, is a rare, lethal and inherited amyloidosis. The disease is caused by mutated transthyretin (TTR). The mutation is characterized by an exchange of valine for methionine at position 30 (ATTRVal30Met). FAP is characterised by progressive polyneuropathy affecting both the peripheral and autonomic nervous system (ANS). Cardiac arrhythmia and autonomic disturbances are common as well as gastrointestinal symptoms: such as constipation and diarrhoea.

Today, orthotopic liver transplantation (LTx) is the only treatment to stop the progression of FAP. The rationale for this is because 95% of TTR is synthesized by the liver, a liver transplantation should abolish the production of new mutated amyloidogenic TTR. The first liver transplantation for FAP was performed in Sweden 1990.

Heart complications and autonomic disturbances are common in FAP patients both before and after liver transplantation. The aim of the present study was three-fold: to determine whether liver transplantation affects the natural course of cardiac arrhythmias and cardiac autonomic function; to predict the risk of ventricular arrhythmias; and to elucidate heart rate variability (HRV) patterns by power spectrum analysis and Poincaré plots.

In total, ninety-seven Swedish FAP patients were included in the studies. The patients underwent 24-hours electrocardiography (Holter) recordings, and/or signal averaged electrocardiography (SAECG) and heart rate variability.

The study showed that many patients developed cardiac arrhythmias and conduction disturbances after LTx. Approximately 25 percent of patients were pacemaker treated after LTx. The SAECG recordings disclosed that many FAP patients had ventricular late potentials (LP) compared with healthy subjects, and that LP were associated with nonsustained ventricular arrhythmia. Analyses of heart rate variability (HRV) showed reduced autonomic function in the majority of patients. Some patients had high HRV with broadband power spectra and Poincaré graphs with a fan or complex pattern. These novel findings could be an indicator of ECG abnormalities (subtle atrial arrhythmia) in FAP patients instead of reflecting normal cardiac autonomic modulation. The HRV studies also showed that LTx preserves cardiac autonomic function in FAP.

In conclusion, cardiac arrhythmias, late potentials and reduced heart rate variability were common in Swedish patients with FAP, whether they underwent liver transplantation or not. The absence of LP may indicate a low risk for ventricular tachycardia in FAP patients.

Place, publisher, year, edition, pages
Umeå: Kirurgisk och perioperativ vetenskap, 2007. 65 p.
Series
Umeå University medical dissertations, ISSN 0346-6612 ; 1116
National Category
Physiology
Identifiers
urn:nbn:se:umu:diva-1407 (URN)978-91-7264-413-7 (ISBN)
Public defence
2007-11-16, Sal 933, Norrlands universitetssjukhus, Umeå, 13:00 (English)
Opponent
Supervisors
Available from: 2007-10-26 Created: 2007-10-26 Last updated: 2009-05-25Bibliographically approved

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CiteExportLink to record
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