ABSTRACT
BACKGROUND: Peripartum cardiomyopathy (PPCM) is a multifactorial disease. Although the specific aetiology and pathogenesis of PPCM are unknown, several hypotheses have been proposed, including selenium deficiency. However, the risk of PPCM from selenium deficiency was not previously quantified. This posthoc analysis of peripartum cardiomyopathy in Nigeria (PEACE) registry data aimed to determine if selenium deficiency is an independent risk factor for PPCM. METHODS: Apparently healthy women who delivered within the previous 8 weeks and PPCM patients in Kano, Nigeria, were compared for selenium deficiency (<70µg/L) and other relevant socio-demographic and clinical characteristics. Selenium level was measured at recruitment for each subject. Independent predictors of PPCM were determined using logistic regression models. RESULTS: 159 PPCM patients and 90 age-matched controls were consecutively recruited. 84.9% of the patients and 3.3% of controls had selenium deficiency. Selenium deficiency independently increased the odds for PPCM by 167-fold while both unemployment and lack of formal education independently increased the odds by 3.4-fold. CONCLUSION: Selenium deficiency was highly prevalent among PPCM patients in Kano, Nigeria, and significantly increased the odds for PPCM. These results could justify screening of women in their reproductive years for selenium deficiency, particularly those living in regions with high incidence of PPCM. The results also call for the setting up of a definitive clinical trial of selenium supplementation in PPCM patients with selenium deficiency, to further define its benefits in the treatment of PPCM.
CONTEXTE: La cardiomyopathie péripartum (CMPP) est une maladie multifactorielle. Bien que l'étiologie spécifique et la pathogenèse de la CMPP soient inconnues, plusieurs hypothèses ont été proposées, notamment la carence en sélénium. Cependant, le risque de CMPP lié à la carence en sélénium n'a pas été précédemment quantifié. Cette analyse post-hoc des données du registre de la cardiomyopathie péripartum au Nigéria (PEACE) visait à déterminer si la carence en sélénium est un facteur de risque indépendant de la CMPP. MÉTHODES: Des femmes apparemment en bonne santé ayant accouché dans les 8 semaines précédentes et des patientes atteintes de CMPP à Kano, au Nigéria, ont été comparées pour la carence en sélénium (<70µg/L) et d'autres caractéristiques socio-démographiques et cliniques pertinentes. Le taux de sélénium a été mesuré au recrutement pour chaque sujet. Les prédicteurs indépendants de la CMPP ont été déterminés à l'aide de modèles de régression logistique. RÉSULTATS: 159 patientes atteintes de CMPP et 90 témoins appariés selon l'âge ont été recrutés consécutivement. 84,9% des patientes et 3,3% des témoins présentaient une carence en sélénium. La carence en sélénium augmentait indépendamment les chances de CMPP de 167 fois, tandis que le chômage et le manque d'éducation formelle augmentaient indépendamment les chances de 3,4 fois. CONCLUSION: La carence en sélénium était très répandue parmi les patientes atteintes de CMPP à Kano, au Nigéria, et augmentait significativement les chances de CMPP. Ces résultats pourraient justifier le dépistage de la carence en sélénium chez les femmes en âge de procréer, en particulier celles vivant dans des régions à forte incidence de CMPP. Les résultats appellent également à la mise en place d'un essai clinique définitif sur la supplémentation en sélénium chez les patientes atteintes de CMPP présentant une carence en sélénium, afin de définir davantage ses avantages dans le traitement de la CMPP. MOTS-CLÉS: Cardiomyopathie Péripartum; Carence en Sélénium; Facteur de Risque.
Subject(s)
Cardiomyopathies , Malnutrition , Selenium , Humans , Female , Peripartum Period , Nigeria/epidemiology , Risk Factors , Cardiomyopathies/epidemiology , Cardiomyopathies/etiologyABSTRACT
BACKGROUND: There are few and conflicting reports in the literature about the relationship between parity and maternal cardiac function. The study aimed to assess the impact of parity on cardiac structure and function in apparently healthy pregnant women in Nigeria. METHODS: This was a cross-sectional study carried out in 3 tertiary centers in Kano, and 1 in Ile-Ife, Nigeria. 112 apparently healthy pregnant women were consecutively recruited between the 28th and 38th weeks of gestation, and their cardiac structure and function assessed using echocardiography. Left ventricular (LV) systolic dysfunction was defined as LV ejection fraction of below 50%, and diastolic dysfunction was graded using mitral filling and tissue Doppler velocities. RESULTS: LV systolic dysfunction and diastolic dysfunction were found in 6 (5.4%) subjects and 20 (17.9%) subjects, respectively. Age (p= <0.0001), left atrial (LA) size (P<0.0001), interventricular septal thickness at end diastole (IVSD) (p= 0.005), posterior wall thickness at end diastole (PWTD) (p=0.004) and QRS duration (p= <0.0001) all increased progressively with higher parity, while tricuspid annular systolic excursion (p=0.320) decreased with higher parity. There was significant positive correlation between parity and age (r= 0.475, p= <0.0001), LA size (r=0.332, p= <0.0001), IVSD (r=0.264, p= 0.005) and PWTD (r= 0.343, p= <0.0001). LV systolic function was not significantly associated with parity. CONCLUSION: Our findings suggested that parity was significantly associated with myocardial remodeling in apparently healthy pregnant women.
CONTEXTE: Il existe peu de données contradictoires dans la littérature sur la relation entre la parité et la fonction cardiaque maternelle. L'étude visait à évaluer l'impact de la parité sur la structure et la fonction cardiaques chez des femmes enceintes apparemment en bonne santé au Nigeria. METHODES: Il s'agissait d'une étude transversale menée dans 3 centres tertiaires à Kano et 1 à Ile-Ife, au Nigeria. 112 femmes enceintes apparemment en bonne santé ont été recrutées consécutivement entre la 28* et la 38* semaine de gestation, et leur structure et fonction cardiaques ont été évaluées par échocardiographie. La dysfonction systolique du ventricule gauche (VG) a été définie comme une fraction d'éjection du VG inférieure à 50 %, et la dysfonction diastolique a été graduée en utilisant le remplissage mitral et les vitesses Doppler tissulaires. RESULTATS: Un dysfonctionnement systolique VG et un dysfonctionnement diastolique ont été trouvés chez 6 (5,4 %) sujets et 20 (17,9 %) sujets respectivement. Âge (p=<0,0001), taille de l'oreillette gauche (LA) (P<0,0001), épaisseur du septum interventriculaire en fin de diastole (IVSD) (p=0,005), épaisseur de la paroi postérieure en fin de diastole (PWTD)(p=0,004) et La durée du QRS (p = <0,0001) a augmenté progressivement avec une parité plus élevée, tandis que l'excursion systolique annulaire tricuspide (p = 0,320) a diminué avec une parité plus élevée. Il y avait une corrélation positive significative entre la parité et l'âge (r = 0,475, p = <0,0001), la taille LA (r = 0,332, p = <0,0001), IVSD (r = 0,264, p = 0,005) et PWTD (r = 0,343, p=<0,0001). La fonction systolique VG était associée à la parité. CONCLUSION: Nos résultats suggèrent que la parité est significativement associée au remodelage du myocarde chez les femmes enceintes apparemment en bonne santé. n'était pas significatif. Mots clés: Grossesse, Parité, Structure Cardiaque, Registre peace.
Subject(s)
Pregnant Women , Ventricular Dysfunction, Left , Female , Humans , Pregnancy , Diastole , Nigeria , Parity , Cross-Sectional StudiesABSTRACT
Background: The relationship between blood pressure (BP) trajectories and outcomes in patients with peripartum cardiomyopathy (PPCM) is not clear. Aim: The study aimed to assess the clinical features and outcomes (all-cause mortality and unrecovered left ventricular [LV] systolic function) of PPCM patients grouped according to their baseline systolic BP (SBP). Patients and Methods: PPCM patients presenting to 14 tertiary hospitals in Nigeria were consecutively recruited between June 2017 and March 2018 and then followed up till March 2019. SBP at first presentation was used to categorize the patients into seven groups: <90, 90-99, 100-109, 110-119, 120-129, 130-139, and ≥140 mmHg. Unrecovered LV systolic function was defined as echocardiographic LV ejection fraction (LVEF) below 55% at the last profiling. Results: Two hundred and twenty-seven patients were recruited and followed up for a median of 18 months. Of these, 4.0% had <90 mmHg, 16.3% had 90-99 mmHg, 24.7% had 100-109 mmHg, 24.7% had 110-119 mmHg, 18.5% had 120-129 mmHg, 7.5% had 130-139 mmHg, and 4.4% had ≥140 mmHg of SBP at presentation. The highest frequency of all-cause mortality was recorded among patients with SBP ≤90 mmHg (30.8%) followed by those with 90-99 mmHg (20.5%) (P = 0.076), while unrecovered LV systolic function did not differ significantly between the groups (P = 0.659). In a Cox proportional regression model for all-cause mortality, SBP <90 mmHg had a hazard ratio (HR) of 4.00 (95% confidence interval [CI] 1.49-10.78, P = 0.006), LVEF had an HR of 0.94 (95% CI 0.91-0.98, P = 0.003, B = 0.06%), and use of angiotensin-converting enzyme or angiotensin receptor and/or ß-receptor blockers had an HR of 1.71 (95% CI 0.93-3.16, P = 0.085). However, SBP was not associated with LV function recovery. Conclusion: In our cohort of PPCM patients, one-fifth was hypotensive at presentation. SBP <90 mmHg at presentation was associated with a four-fold higher risk of all-cause mortality during a median follow-up of 18 months.
Subject(s)
Cardiomyopathies , Peripartum Period , Humans , Blood Pressure , Ventricular Function, Left , Stroke VolumeABSTRACT
AIMS: The aim of this study was to describe the incidence, clinical characteristics and risk factors of peripartum cardiomyopathy (PPCM) in Nigeria. METHODS AND RESULTS: The study was conducted in 22 hospitals in Nigeria, and PPCM patients were consecutively recruited between June 2017 and March 2018. To determine factors associated with PPCM, the patients were compared with apparently healthy women who recently delivered, as controls. Four hundred six patients were compared with 99 controls. The incidence and disease burden (based on the rate of consecutive recruitment of subjects) varied widely between the six geographical zones of Nigeria. From the North-West zone, 72.3% of the patients was recruited, where an incidence as high as 1 per 96 live births was obtained in a centre, while the disease was uncommon (7.6% of all recruited patients) in the South. Majority of the patients (76.6%) and controls (74.8%) (p = 0.694) were of Hausa-Fulani ethnic group. Atrial fibrillation, intracardiac thrombus, stroke, and right ventricular systolic dysfunction were found in 1.7%, 6.4%, 2.2%, and 54.9% of the patients, respectively. Lack of formal education (odds ratio [OR] 3.08, 95% confidence interval [1.71, 5.53]; P < 0.001), unemployment (OR: 3.28 [2.05, 5.24]; P < 0.001), underweight (OR: 13.43 [4.17, 43.21]; P < 0.001) and history of pre-eclampsia (OR: 9.01 [2.18, 37.75]; P = 0.002) emerged as independent PPCM risk factors using regression models. Customary hot baths (OR: 1.24 [0.80, 1.93]; P = 0.344), pap enriched with dried lake salt (OR: 1.20 [0.74, 1.94]; P = 0.451), and Hausa-Fulani ethnicity (OR: 1.11 [0.67, 1.84]; P = 0.698) did not achieve significance as PPCM risk factors. CONCLUSIONS: In Nigeria, the burden of PPCM was greatest in the North-West zone, which has the highest known incidence. PPCM was predicted by sociodemographic factors and pre-eclampsia, which should be considered in its control at population level. Postpartum customary birth practices and Hausa-Fulani ethnicity were not associated with PPCM in Nigeria.
Subject(s)
Cardiomyopathies/epidemiology , Peripartum Period , Pregnancy Complications, Cardiovascular/epidemiology , Registries , Adult , Cardiomyopathies/physiopathology , Female , Follow-Up Studies , Hemodynamics/physiology , Humans , Nigeria/epidemiology , Pregnancy , Pregnancy Complications, Cardiovascular/physiopathology , Retrospective Studies , Risk FactorsABSTRACT
PMP22 is a transmembrane glycoprotein component of myelin, important for myelin functioning. Mutation of PMP22 gene which encodes for the production of PMP22 glycoprotein is associated with a variety of inherited neuropathies. This literature review sought to review the molecular mechanism and clinical features of inherited neuropathies caused by PMP22 duplication. PMP22 duplication causes CMT1A which accounts for more than half of all CMT cases and about 70% of CMT1 cases. It manifests with muscle weakness, depressed reflexes, impaired distal sensation, hand and foot deformities, slowing of NCV and onion bulbs. With no specific treatment available, it is managed conservatively. Future treatment may be based on the molecular genetics of the disease.
Subject(s)
Charcot-Marie-Tooth Disease , Gene Duplication/genetics , Myelin Proteins/genetics , Charcot-Marie-Tooth Disease/genetics , Charcot-Marie-Tooth Disease/pathology , Charcot-Marie-Tooth Disease/physiopathology , DNA Mutational Analysis , Electrophysiology , Humans , Models, Molecular , NeuroimagingABSTRACT
We report a rare case of common atrium and acute decompensated heart failure most likely precipitated by acute bacterial pericarditis leading to premature death, in a 25-year-old male footballer. The silent course of the disease for decades as well as the diagnostic and management pitfalls of this case illustrates the importance of early detection by echocardiography and urgent appropriate treatment in intensive care settings to limit the poor prognosis of the condition.