Povezava med izraženostjo nekompaktnega miokarda in ishemijo srčne mišice pri bolnikih z izolirano nekompakcijsko kardiomiopatijo

Cerar, Andraž (author), Koželj, Mirta (mentor)

Izhodišče: Nekompakcijska kardiomiopatija (NKMP) je prirojena bolezen srca, ki jo označuje zastoj v procesu kompakcije v zgodnjem gestacijskem obdobju. Proces kompakcije je pomemben tudi za razvoj koronarne mikrocirkulacije, vendar doslej spremembe v delovanju srca pri bolnikih z NKMP vzročno še niso bile opredeljene. Namen naše raziskave je bil opredeliti morebitno korelacijo med ishemijo miokarda in stopnjo srčnega popuščanja pri bolnikih z NKMP. Metode: V prospektivno raziskavo smo vključili 41 bolnikov z NKMP (28 moških in 13 žensk), v starosti med 21 in 70 let. Diagnoza NKMP je bila potrjena s srčno magnetno resonanco. Pri bolnikih smo opravili ultrazvočno preiskavo srca, kjer smo določili iztisni delež levega prekata (LVEF), končni diastolični volumen levega prekata (LVEDV), ocenili polnilne tlake levega prekata (E/e’), izmerili bazalni premer desnega prekata (RVId), longitudinalno gibanje trikuspidalnega obroča (TAPSE), delež spremembe površine desnega prekata (FAC) ter izračunali globalno vzdolžno sistolično deformacijo (GLS). Izmerili smo izhodiščno serumsko vrednost natriuretičnega peptida (NT-proBNP). Z enofotonsko emisijsko računalniško tomografijo (angl. single photon emission computerized tomography, SPECT) miokarda v mirovanju in ob obremenitvi smo ocenili morebitno pomembno ishemijo miokarda, ki smo jo opredeli kot razliko v scintigramih srca v mirovanju in ob obremenitvi (angl. summed difference score, SDS) ? 2. Rezultati: Glede na SDS smo razdelili bolnike z NKMP v dve skupini, ki se med seboj nista razlikovali v spolu, starosti, jetrnem ali ledvičnem delovanju. Ishemijo miokarda smo dokazali pri 11 bolnikih (27 %, skupina A), 30 bolnikov ni imelo dokazane pomembne ishemije miokarda (73 %, skupina B). V primerjavi s skupino B je imela skupina A statistično pomembno nižji LVEF (35 ± 15 % skupina A in 53 ± 11 % skupina B, P<0,001), večji LVEDV (188 ± 52 mL in 136 ± 52 mL, P = 0,007), višje polnilne tlake, ocenjene z E/e’ (17,2 ± 14,3 in 10,2 ± 5,2, P = 0,029), večji RVId (3,3 ± 0,6 cm in 2,9 ± 0,5 cm, P = 0,016), nižji TAPSE (1,9 ± 0,6 cm in 2,5 ± 0,4 cm, P < 0,001), nižji FAC (30,7 ± 7,7 % in 44,9 ± 10,2 %, P < 0,001), nižji GLS (-9,9 ± 5,2 % in -14,5 ± 4,1 %, P = 0,001) in višje serumske vrednosti NT-proBNP (1691 ± 1883 pg/mL in 422 ± 877 pg/mL, P = 0,006). Opazili smo tudi korelacijo med večjim SDS in nižjim LVEF (r = -0,48, P=0,001), večjim LVEDV (r = 0,39, P = 0,012), večjim RVId (r = 0,41, P=0,032), nižjim TAPSE (r = -0,49, P = 0,008), nižjim FAC (r = -0,51, P = 0,006), nižjim GLS (r = 0,352, P = 0,024) in višjimi vrednostmi serumskega NT-proBNP (r = 0,66, P < 0,001). Zaključek: Ishemija miokarda, dokazana s scintigrafijo miokarda, je pri bolnikih z NKMP povezana s stopnjo srčnega popuščanja. Bolniki z NKMP, ki so imeli dokazano ishemijo miokarda, so imeli značilno večje dimenzije in slabši iztisni delež tako levega kot desnega prekata ter višje ocenjene polnilne tlake levega prekata v primerjavi z bolniki brez jasne ishemije miokarda. Bolniki z ishemijo miokarda so imeli tudi bolj izraženo delovanje nevrohumoralnega sistema, ocenjenega s serumsko vrednostjo NT-proBNP.

• Language: Slovenian
• Keywords: nekompakcijska kardiomiopatija, ishemija srčne mišice, srčno popuščanje
• Work type: Doctoral dissertation
• Organization: MF - Faculty of Medicine
• Year: 2021
• PID: 20.500.12556/RUL-125311
• COBISS.SI-ID: 57520899
• Publication date in RUL: 10.03.2021

Secondary language

• Language: English
• Title: Association between the extent of noncompacted myocardium and heart muscle ischemia in the patients with isolated noncompaction cardiomyopathy

Abstract

Background: Noncompaction cardiomyopathy (NCM) is a congenital heart disease characterized by an arrest of the myocardial compaction process in the early gestational period. As the process is thought to be responsible for the formation of coronary microvasculature, the functional impact of these changes in patients with NCM remains undefined. The aim of our study was to analyse a potential correlation between myocardial ischemia and heart failure severity in NCM patients. Methods: In this prospective study, we enrolled 41 NCM patients (28 male, 13 female), aged 21 to 70 years. NCM diagnosis had to be verified by cardiac magnetic resonance imaging. Echocardiography has been performed in all patients, defining left ventricle ejection fraction (LVEF), left ventricle end-diastolic volume (LVEDV), estimating left ventricle filling pressures (E/e’), measuring right ventricle inflow diastolic diameter (RVId), tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC) as well as calculating global longitudinal strain (GLS) with post-processing. Serum levels of NT-proBNP have been measured at baseline. Myocardial single-photon emission computed tomography (SPECT) at rest and on stress was used to define significant myocardial ischemia, defined as summed difference score (SDS) ⡥2. Results: According to SDS the patients have been divided in two groups, which did not differ in sex, age, kidney or liver function. Myocardial ischemia has been demonstrated in 11 patients (27%, Group A), 30 patients showed no significant myocardial ischemic changes (73%, Group B). When compared to Group B, Group A had significantly lower LVEF (35±15% in Group A vs. 53±11% in Group B, P<0.001), higher LVEDV (188±52mL vs. 136±52mL, P=0.007), higher filling pressured estimated by E/e’ ratio (17.2±14.3 in 10.2±5.2, P=0.029), higher RVId (3.3±0.6cm vs. 2.9±0.5cm, P=0.016), lower TAPSE (1.9±0.6cm vs. 2.5±0.4cm, P<0.001), lower FAC (30.7±7.7% vs. 44.9±10.2%, P<0.001), lower GLS (-9.9±5.2% vs. -14.5±4.1%, P=0.001) and higher serum NT-proBNP levels (1691±1883pg/mL vs. 422±877pg/mL, P=0.006). Overall, higher SDS was associated with lower LVEF (r=-0.48, P=0.001), higher LVEDV (r=0.39, P=0.012), higher RVId (r=0.41, P=0.032), lower TAPSE (r=-0.49, P=0.008), lower FAC (r=-0.51, P=0.006), lower GLS (r=0.352, P=0.024) and higher serum levels of NT-proBNP (r=0.66, P<0.001). Conclusion: The presence of myocardial ischemia in patients with NCM is associated with heart failure severity. NCM patients with proven myocardial ischemia had significantly enlarged dimensions and a worse ejection fraction of both left and right ventricle, as well as higher estimated left ventricle filling pressures, compared to the patients without myocardial ischemia. Patients with myocardial ischemia also had more pronounced neurohumoral activation, assessed by serum levels of NT-proBNP.

• Keywords: noncompaction cardiomyopathy, myocardial ischemia, heart failure