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The Journal of Thoracic and... Jun 2024To evaluate the outcomes of the extracardiac (EC) Fontan operation at a single institution.
OBJECTIVE
To evaluate the outcomes of the extracardiac (EC) Fontan operation at a single institution.
METHODS
We conducted a retrospective study of 398 patients from a single institution who underwent their initial EC Fontan operation between 1997 and 2020. We determined the incidence of, and risk factors for, failure of the Fontan circulation (which includes death, Fontan takedown, heart transplantation, protein losing enteropathy, plastic bronchitis and functional status at the last follow up).
RESULTS
The median follow-up time was 10.3 (IQR 6.4-14.6) years. The overall survival rate was 96% and 86% at 10 and 20 years after EC Fontan operation. There were 6 early (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation at 10 years was 88% and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular (RV) dominance (hazard ratio [HR], 4.7; P < 0.001; 95% confidence interval [CI], 2.1-10.5), aortic atresia (HR 5.5; P < 0.001; 95% CI, 2.3-12.8) and elevated mean pulmonary artery (PA) pressure (HR, 2.3; P = 0.002; 95% CI, 1.2-6.7).
CONCLUSIONS
Rates of failure of the Fontan circulation are low following the contemporary EC Fontan operation. Risk factors for failure of the EC Fontan circulation include RV dominance, aortic atresia and elevated PA pressures.
PubMed: 38897544
DOI: 10.1016/j.jtcvs.2024.05.030 -
The American Journal of Cardiology Jun 2024Impaired pulmonary circulation hemodynamics are characteristic of pulmonary hypertension (PH). We therefore sought to evaluate possible correlations between endothelial...
Impaired pulmonary circulation hemodynamics are characteristic of pulmonary hypertension (PH). We therefore sought to evaluate possible correlations between endothelial function noninvasively assessed by flow-mediated dilation (FMD) and hemodynamic parameters at right-sided cardiac catheterization in patients with clinically suspected PH. Consecutive outpatients with suspected PH were enrolled in the study. In all patients, endothelial function was assessed by FMD and hemodynamic parameters (pulmonary artery pressure [PAP]); pulmonary vascular resistances [PVR]) were derived by right-sided cardiac catheterization. For this study, 95 consecutive patients with suspected PH were enrolled (mean age 63 ± 13 years, 58% male) and included in the analysis. FMD values were significantly correlated with systolic (s)PAP levels (r = -0.29, p = 0.016); correlation with PVR was of borderline significance (r = -0.21, p = 0.78). After multivariable regression analysis including age, gender, tricuspid annular plane systolic excursion and peak tricuspid regurgitation velocity (peak TRV), and FMD, the latter remained significantly correlated with systolic pulmonary artery pressure (sPAP) values (B = -47, p = 0.02). After classifying patients according to median levels of peak TRV and FMD into 3 groups (neither, either, or both impaired), progressively increased levels of sPAP, mean PAP, and PVR were found (p for trend <0.001 in all cases). FMD values were inversely related to sPAP levels in a small population of patients with clinically suspected PH. In combination with peak TRV levels, FMD values noninvasively assessed were predictive of increased sPAP, mean PAP, and PVR.
PubMed: 38897266
DOI: 10.1016/j.amjcard.2024.06.019 -
Journal of Clinical Medicine May 2024Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital... (Review)
Review
Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital heart disease seen in adult patients is the Fontan circulation. These patients have complex physiology and are at risk for several complications, including thrombosis of the Fontan pathway, pulmonary vascular disease, heart failure, atrial arrhythmias, atrioventricular valve regurgitation, and protein-losing enteropathy. This review discusses the commonly encountered phenotypes of Fontan circulatory failure and their contemporary management.
PubMed: 38892760
DOI: 10.3390/jcm13113049 -
Animal Models and Experimental Medicine Jun 2024Under hypoxia, exaggerated compensatory responses may lead to acute mountain sickness. The excessive vasodilatory effect of nitric oxide (NO) can lower the hypoxic...
BACKGROUND
Under hypoxia, exaggerated compensatory responses may lead to acute mountain sickness. The excessive vasodilatory effect of nitric oxide (NO) can lower the hypoxic pulmonary vasoconstriction (HPV) and peripheral blood pressure. While NO is catalyzed by various nitric oxide synthase (NOS) isoforms, the regulatory roles of these types in the hemodynamics of pulmonary and systemic circulation in living hypoxic animals remain unclear. Therefore, this study aims to investigate the regulatory effects of different NOS isoforms on pulmonary and systemic circulation in hypoxic rats by employing selective NOS inhibitors and continuously monitoring hemodynamic parameters of both pulmonary and systemic circulation.
METHODS
Forty healthy male Sprague-Dawley (SD) rats were randomly divided into four groups: Control group (N-nitro-D-arginine methyl ester, D-NAME), L-NAME group (non-selective NOS inhibitor, N-nitro-L-arginine methyl ester), AG group (inducible NOS inhibitor group, aminoguanidine), and 7-NI group (neurological NOS inhibitor, 7-nitroindazole). Hemodynamic parameters of rats were monitored for 10 min after inhibitor administration and 5 min after induction of hypoxia [15% O, 2200 m a. sl., 582 mmHg (76.5 kPa), Xining, China] using the real-time dynamic monitoring model for pulmonary and systemic circulation hemodynamics in vivo. Serum NO concentrations and blood gas analysis were measured.
RESULTS
Under normoxia, mean arterial pressure and total peripheral vascular resistance were increased, and ascending aortic blood flow and serum NO concentration were decreased in the L-NAME and AG groups. During hypoxia, pulmonary arterial pressure and pulmonary vascular resistance were significantly increased in the L-NAME and AG groups.
CONCLUSIONS
This compensatory mechanism activated by inducible NOS and endothelial NOS effectively counteracts the pulmonary hemodynamic changes induced by hypoxic stress. It plays a crucial role in alleviating hypoxia-induced pulmonary arterial hypertension.
PubMed: 38888011
DOI: 10.1002/ame2.12453 -
Pulmonary Circulation Apr 2024Parameters of cardiopulmonary exercise testing significantly discriminate between healthy subjects and patients with pulmonary hypertension (PH), also according to the...
Parameters of cardiopulmonary exercise testing significantly discriminate between healthy subjects and patients with pulmonary hypertension (PH), also according to the new 2022 definition of pulmonary hypertension (mean pulmonary arterial pressure mPAP > 20 mmHg). The cut-offs indicating on PH were peakVO ≤ 16.7 mL/min/kg (Youden-Index YI = 0.79), pCO@AT ≤ 34 mmHg (YI = 0.67), and VE/VCO@AT ≤ 30 (YI = 0.76).
PubMed: 38887743
DOI: 10.1002/pul2.12398 -
Pulmonary Circulation Apr 2024Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension...
Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.
PubMed: 38887742
DOI: 10.1002/pul2.12395 -
Pulmonary Circulation Apr 2024Pulmonary hypertension (PH) is associated with adverse outcomes in chronic kidney disease (CKD) patients. Our study suggests mildly elevated pulmonary vascular...
Association of mildly elevated pulmonary vascular resistance with major cardiovascular events in pulmonary hypertension and chronic kidney disease: A retrospective cohort analysis.
Pulmonary hypertension (PH) is associated with adverse outcomes in chronic kidney disease (CKD) patients. Our study suggests mildly elevated pulmonary vascular resistance ( > 2 to ≤ 3) is independently associated with major adverse cardiovascular events at 1-year follow-up. Early diagnosis of precapillary PH in CKD patients can potentially improve clinical outcomes.
PubMed: 38887741
DOI: 10.1002/pul2.12401 -
Circulation Jun 2024
Topics: Humans; Heart Failure; Glucosides; Benzhydryl Compounds; Stroke Volume; Sodium-Glucose Transporter 2 Inhibitors; Male; Hemodynamics; Female; Body Composition; Aged; Middle Aged
PubMed: 38885297
DOI: 10.1161/CIRCULATIONAHA.124.069479 -
Advances in Experimental Medicine and... 2024In normal cardiovascular development in birds and mammals, the outflow tract of the heart is divided into two distinct channels to separate the oxygenated systemic blood... (Review)
Review
In normal cardiovascular development in birds and mammals, the outflow tract of the heart is divided into two distinct channels to separate the oxygenated systemic blood flow from the deoxygenated pulmonary circulation. When the process of outflow tract septation fails, a single common outflow vessel persists resulting in a serious clinical condition known as persistent truncus arteriosus or common arterial trunk. In this chapter, we will review molecular pathways and the cells that are known to play a role in the formation and development of the outflow tract and how genetic manipulation of these pathways in animal models can result in common arterial trunk.
Topics: Animals; Humans; Disease Models, Animal; Signal Transduction; Truncus Arteriosus; Truncus Arteriosus, Persistent
PubMed: 38884754
DOI: 10.1007/978-3-031-44087-8_52 -
Advances in Experimental Medicine and... 2024The great arteries of the vertebrate carry blood from the heart to the systemic circulation and are derived from the pharyngeal arch arteries. In higher vertebrates, the... (Review)
Review
The great arteries of the vertebrate carry blood from the heart to the systemic circulation and are derived from the pharyngeal arch arteries. In higher vertebrates, the pharyngeal arch arteries are a symmetrical series of blood vessels that rapidly remodel during development to become the asymmetric aortic arch arteries carrying oxygenated blood from the left ventricle via the outflow tract. At the base of the aorta, as well as the pulmonary trunk, are the semilunar valves. These valves each have three leaflets and prevent the backflow of blood into the heart. During development, the process of aortic arch and valve formation may go wrong, resulting in cardiovascular defects, and these may, at least in part, be caused by genetic mutations. In this chapter, we will review models harboring genetic mutations that result in cardiovascular defects affecting the great arteries and the semilunar valves.
Topics: Animals; Aorta, Thoracic; Humans; Mutation; Disease Models, Animal; Aortic Valve; Heart Defects, Congenital
PubMed: 38884748
DOI: 10.1007/978-3-031-44087-8_46