Pacemaker implantation in familial amyloid polyneuropathy: when and for whom?

Milner, James; Teixeira, Rafaela Nicolau; Marinho, André; Silva, Nuno; Calretas, S.; Ferrão, José; Furtado, Emanuel; Telo, M; Ventura, Miguel; Cristóvão, João; Elvas, Luís; Pego, Guilherme Mariano; António, Natália

doi:10.1007/s10840-019-00517-y

Cited by 9 publications

(5 citation statements)

References 13 publications

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“…Although cardiac pacing can be helpful in symptomatic relief, there are no significant beneficial effects on patient survival. [62][63][64][65] Moreover, ventricular dyssynchrony or leadinduced tricuspid regurgitation caused by right ventricular pacing might have deleterious impacts on haemodynamics.…”

Section: Pacemaker Implantationmentioning

confidence: 99%

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

Yamamoto¹,

2019

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Transthyretin cardiac amyloidosis (ATTR-CA) demonstrates progressive, potentially fatal, and infiltrative cardiomyopathy caused by extracellular deposition of transthyretin-derived insoluble amyloid fibrils in the myocardium. Two distinct types of transthyretin (wild type or variant) become unstable, and misfolding forms aggregate, resulting in amyloid fibrils. ATTR-CA, which has previously been underrecognized and considered to be rare, has been increasingly recognized as a cause of heart failure with preserved ejection fraction among elderly persons. With the advanced technology, the diagnostic tools have been improving for cardiac amyloidosis. Recently, the efficacy of several disease-modifying agents focusing on the amyloidogenic process has been demonstrated. ATTR-CA has been changing from incurable to treatable. Nevertheless, there are still no prognostic improvements due to diagnostic delay or misdiagnosis because of phenotypic heterogeneity and comorbidities. Thus, it is crucial for clinicians to be aware of this clinical entity for early diagnosis and proper treatment. In this mini-review, we focus on recent advances in diagnosis and treatment of ATTR-CA.

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Section: Pacemaker Implantationmentioning

confidence: 99%

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

Yamamoto¹,

2019

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“…There are discrepancies in implementation of preoperative pacemaker insertion in FAP patients with cardiac involvement who are undergoing LT [ 14 ]. After in-depth discussion with cardiologists and surgeons, we decided to omit temporary pacemaker insertion and proceeded with the procedure using an external patch.…”

Section: Discussionmentioning

confidence: 99%

Domino living donor liver transplantation of familial amyloid polyneuropathy patient - A case report -

et al. 2020

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Background: Familial amyloid polyneuropathy (FAP) is caused by mutation in a gene transcribing transport protein produced mainly by the liver. Liver transplantation is required to stop FAP progression, but the pathology causes anesthetic management challenges.Case: We report a case of domino living donor liver transplantation in an FAP patient. No intraoperative events occurred; however, during postoperative day 1 in the intensive care unit (ICU), the FAP patient underwent multiple cardiopulmonary resuscitation (CPR) sessions due to pulseless electrical activity following a sudden drop in blood pressure and ventricular tachycardia. Despite ICU management, the patient died after the third CPR session.Conclusions: Various anesthetic management techniques should be considered for FAP patients. Anesthetic management was carefully assessed with the use of isoflurane, isoproterenol, and an external patch. The cause of deterioration in the ICU is unclear, but further investigation is needed to prevent and better manage postoperative morbidity and mortality.

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“…Data did not suggest improved mortality or morbidity in patients who received a PPM before liver transplantation for prophylaxis. 37 A recent study by Porcari et al noted that 8.9% of patients with the diagnosis of CA needed PPM implantation within 3 years of diagnosis. 38 Thus, until further data is available, it is reasonable to reserve pacemaker placement for secondary prevention.…”

Section: Diagnosismentioning

confidence: 99%

A review of recent advances in the diagnosis of cardiac amyloidosis, treatment of its cardiac complications, and disease-modifying therapies

Raval

Siddiq²,

Sharma³

et al. 2023

F1000Res

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Cardiac amyloidosis (CA), a significant condition resulting in infiltrative cardiomyopathy and heart failure with preserved ejection fraction (HFpEF), is caused by extracellular deposition of amyloid fibrils in the heart. Even though this has been known for an extended period, its prevalence in elderly patients with heart failure is increasingly being recognized. Recent advances in diagnosis with non-invasive methods like technetium pyrophosphate-labeled cardiac scintigraphy (i.e., Tc-PYP scan) and treatment options with tafamidis have played a pivotal role in awareness of the burden of this disease. Management of cardiac complications like heart failure, atrial arrhythmias, conduction block, ventricular arrhythmias, coronary artery disease, and aortic stenosis is now more critical than ever. We aim to review and outline the recent advances in diagnoses of CA. We also review management strategies for cardiac complications of CA with a brief summary of disease-modifying therapies.

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Pacemaker implantation in familial amyloid polyneuropathy: when and for whom?

Cited by 9 publications

References 13 publications

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

Domino living donor liver transplantation of familial amyloid polyneuropathy patient - A case report -

A review of recent advances in the diagnosis of cardiac amyloidosis, treatment of its cardiac complications, and disease-modifying therapies

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