Echocardiographic markers of left ventricular hypertrophy and concentric remodeling – limitations in diagnostics of cardiac amyloidosis, Fabry disease and hypertrophic cardiomyopathy

• Authors: Judyta Znamirowska , Mateusz Tometczak , Justyna Topa , Patrycja Pastor , Damian Waksmundzki , Adam Grzebinoga , Katarzyna Mizia-Stec

Title variants

PL

Echokardiograficzne parametry przerostu lewej komory i remodelingu koncentrycznego – ograniczenia w diagnostyce amyloidozy serca, choroby Fabry’ego i kardiomiopatii przerostowej

Abstracts

PL

WPROWADZENIE: Przerost lewej komory (left ventricular hypertrophy – LVH) jest istotną cechą powiązaną ze wzrostem ryzyka nagłej śmierci sercowej. Typowe przyczyny przerostu obejmujące amyloidozę serca (cardiac amyloidosis – CA), chorobę Fabry’ego (Fabry disease – FD) oraz kardiomiopatię przerostową (hypertrophic cardiomyopathy – HCM) wymagają specyficznego podejścia i rokują niepomyślnie. Echokardiografia przezklatkowa (transthoracic echocardiography – TTE) pozostaje wówczas podstawową metodą diagnostyczną.
MATERIAŁ I METODY: Wykonano jednoośrodkową analizę retrospektywną obejmującą 86 pacjentów z przero-stem ściany lewej komory (left ventricular – LV) stwierdzonym w TTE. Kryteriami włączenia były grubość przegrody międzykomorowej (interventricular septum – IVS) powyżej 10 mm u mężczyzn i 9 mm u kobiet oraz potwierdzona diagnoza CA, FD lub HCM. Badana populacja została podzielona na trzy grupy w zależno-ści od końcowej diagnozy: CA (13 pacjentów), FD (7 pacjentów) oraz HCM (66 pacjentów). Analizowano in-deks masy lewej komory (LV mass index – LVMI), względną grubość ściany lewej komory (relative wall thickness – RWT) oraz typ przerostu.
WYNIKI: LVMI powyżej normy obserwowano u 90,9% pacjentów z CA, wszystkich z FD, 89,5% z HCM. RWT powyżej normy obserwowano u 92,3% pacjentów z CA, 57,1% z FD, 92,4% z HCM. Przerost koncentryczny zdiagnozowano u 75% pacjentów z CA, 57,1% z FD, 84,2% z HCM; przerost ekscentryczny zaobserwowano u 8,3% pacjentów z CA, 42,9% z FD, 5,3% z HCM (p = 0,01). IVS/PWT powyżej normy stwierdzono u 23,1% pacjentów z CA, 28,6% z FD, 79,7% z HCM (p = 0,00001).
WNIOSKI: Mimo iż przerost serca jest cechą typową, nie występuje u wszystkich osób z CA, FD, HCM. Do scharakteryzowania konkretnych chorób, jak CA, FD, HCM, wymagana jest dokładniejsza analiza obejmująca typ przerostu oraz remodeling lewego przedsionka. Na podstawie wyników przerost asymetryczny jest bardziej swoisty dla HCM.

EN

INTRODUCTION: Left ventricular hypertrophy (LVH) is a relevant sign associated with an increased risk of sudden death. The causes of LVH including cardiac amyloidosis (CA), Fabry disease (FD), hypertrophic cardiomyopathy (HCM) are associated with an inauspicious prognosis. Transthoracic echocardiography (TTE) remains the first-step baseline diagnostic method.
MATERIAL AND METHODS: A retrospective one-center analysis of 86 patients (pts) with increased left ventricular (LV) wall thickness in TTE was performed. The inclusion criteria were interventricular septum (IVS) above 10 mm in males, 9 mm in females and the final diagnosis of CA, FD or HCM. The study population was divided into three subgroups: CA (13 pts), FD (7 pts), HCM (66 pts). The LV mass index (LVMI), relative wall thickness (RWT) and type of remodeling were analyzed.
RESULTS: Increased LVMI occurred in 90.9% pts with CA, all with FD, 89.5% with HCM.RWT exceeded the normal range among 92.3% pts with CA, 57.1% with FD, 92.4% with HCM. Concentric hypertrophy was diagnosed in 75% pts with CA, 57.1% with FD, 84.2% with HCM and eccentric in 8.3% pts with CA, 42.9% with FD, 5.3% with HCM (p = 0.01). An abnormal IVS/PWT index was observed in 23.1% pts with CA, 28.6% with FD, 79.7% with HCM (p = 0.00001).
CONCLUSIONS: Although cardiac hypertrophy is a typical sign, it does not occur in all subjects with CA, FD, HCM. More detailed analysis including the form of hypertrophy as well as left atrium remodeling are required to be characterized for specific diseases: CA, FD, HCM. Asymmetrical hypertrophy is more specific for HCM.

Keywords

EN

echocardiography; hypertrophy; remodeling; LVMI; RTW; hypertrophic cardiomyopathy; amyloidosis; Fabry disease

PL

echokardiografia; przerost; remodeling; LVMI; RWT; kardiomiopatia przerostowa; amyloidoza; choroba Fabry’ego

• Publisher: Śląski Uniwersytet Medyczny w Katowicach
• Journal: Annales Academiae Medicae Silesiensis
• Year: 2024
• Issue: 78
• Pages: 219-225

Dates

published

2024

Contributors

author

Judyta Znamirowska

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0009000851030193

author

Mateusz Tometczak

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0009000030429455

author

Justyna Topa

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0009000324290962

author

Patrycja Pastor

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0009000999744520

author

Damian Waksmundzki

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0000000274143771

author

Adam Grzebinoga

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0009000159837564

author

Katarzyna Mizia-Stec

• 1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland

• https://orcid.org/0000000169072799

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Identifiers

DOI

10.18794/aams/189128

Biblioteka Nauki

50376135