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1.
Echocardiography ; 41(6): e15853, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38853623

ABSTRACT

BACKGROUND: Systemic Sclerosis (SSc), an intricate autoimmune disease causing tissue fibrosis, introduces cardiovascular complexities, notably pulmonary hypertension (PH), affecting both survival and quality of life. This study centers on evaluating echocardiographic parameters and endothelial function using flow-mediated dilatation (FMD) in SSc patients, aiming to differentiate those with and without pulmonary arterial hypertension (PAH). The emphasis lies in early detection, given the heightened vulnerability of the right ventricle (RV) in the presence of PH. METHODS: Fifty-nine SSc patients and 48 healthy subjects participated, undergoing clinical examinations, echocardiography, FMD assessments, blood analyses, and right heart catheterization (RHC) according to the ESC/ERS guidelines for diagnosis and treatment of PH. RESULTS: SSc-PAH patients displayed lower FMD, higher frequency of TAPSE < 18 mm, RA area > 18 cm2, act RVOT < 105 ms and TRV > 280 cm/s compared to those without PAH and healthy controls. Resting resistivity index (RI) was higher in SSc patients, with no significant difference between those with and without PAH. Lower FMD% serves as a predictive marker for adverse cardiovascular outcomes in both SSc and SSc-PAH patients. Stratification by TRV levels and PAH presence reveals notable FMD% variations, emphasizing its potential utility. CONCLUSIONS: Early identification of endothelial dysfunction and impaired RV echocardiographic parameters, such as TAPSE and TRV, could aid in predicting right ventricular dysfunction and PAH in SSc patients.


Subject(s)
Echocardiography , Scleroderma, Systemic , Humans , Female , Male , Scleroderma, Systemic/complications , Scleroderma, Systemic/physiopathology , Middle Aged , Echocardiography/methods , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/diagnostic imaging , Adult
3.
J Cardiothorac Surg ; 19(1): 319, 2024 Jun 05.
Article in English | MEDLINE | ID: mdl-38840135

ABSTRACT

BACKGROUND: Aberrant left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect. Its coexistence with patent ductus arteriosus (PDA) is extremely rare. The high pressures created by the left-to-right shunt in the pulmonary arteries can delay symptoms and create a real challenge in diagnosing ALCAPA. Missing this diagnosis can have severe results, including extensive ischemia and sudden death. CASE PRESENTATION: We present a case of an infant born with a large PDA. Initially treated conservatively, however, due to congestive heart failure and lack of weight gain, she underwent surgical ligation of the PDA at the age of four and a half months. Following surgery, she developed pulmonary edema. Echocardiography revealed decreased ventricular function. ECG revealed ST elevations on lateral leads, and serum troponin was significantly increased. The patient underwent cardiac magnetic resonance imaging (MRI), which revealed signs of wall ischemia and decreased function of the left ventricle (LV) with unclear coronary anatomy. Diagnostic catheterization revealed an ALCAPA. She underwent surgical intervention, and the left coronary artery was re-implanted in the aortic sinus. Follow-up revealed slow improvement of cardiac function. DISCUSSION AND LITERATURE REVIEW: The coexistence of PDA and ALCAPA is a very rare occurrence. We found at least 10 reported cases in the literature. Delayed diagnosis might be detrimental. The prognosis of these patients is variable. CONCLUSION: An unusual post-surgical course following PDA repair requires a high index of suspicion and appropriate evaluation for ALCAPA, preferably with angiography.


Subject(s)
Ductus Arteriosus, Patent , Pulmonary Artery , Humans , Ductus Arteriosus, Patent/surgery , Ductus Arteriosus, Patent/complications , Ductus Arteriosus, Patent/diagnosis , Ductus Arteriosus, Patent/diagnostic imaging , Female , Pulmonary Artery/abnormalities , Pulmonary Artery/surgery , Pulmonary Artery/diagnostic imaging , Infant , Coronary Vessel Anomalies/surgery , Coronary Vessel Anomalies/diagnosis , Coronary Vessel Anomalies/complications , Coronary Vessel Anomalies/diagnostic imaging , Echocardiography , Coronary Vessels/surgery , Coronary Vessels/diagnostic imaging , Bland White Garland Syndrome/surgery , Bland White Garland Syndrome/diagnosis , Bland White Garland Syndrome/complications
4.
PLoS One ; 19(6): e0302200, 2024.
Article in English | MEDLINE | ID: mdl-38843270

ABSTRACT

BACKGROUND AND AIMS: Pericardial effusion (PE) is a prevalent form of pericardial involvement in chronic kidney disease (CKD). This study aims to investigate the clinical and laboratory features associated with PE severity in patients with CKD. METHODS: In this cross-sectional study, we examined the medical records of patients admitted to tertiary hospitals with International Classification of Diseases 10th Revision (ICD-10) codes associated with CKD and PE. We included 112 CKD patients in stage 4 and 5 non-dialysis (ND) with PE for assessing the clinical and laboratory features of severity. RESULTS: Patients were divided into two categories based on the severity of PE. Seventy-two patients had mild and 40 had moderate and severe PE. Univariate analysis of demographic and laboratory features on the date of admission demonstrated that chest pain, dyspnea, serum albumin, and neutrophil-to-lymphocyte ratio (NLR) are associated with the severity of PE. The univariate analysis on the date of echocardiography showed significantly higher white blood cell count (WBC), neutrophil count (percentage and absolute count), and NLR, along with significantly lower lymphocyte percentage and serum albumin among patients with moderate and severe PE. In the multivariable analysis of laboratory features, on admission hypoalbuminemia (p-value = 0.014, OR = 4.03, CI: 1.32-12.25) and NLR greater than 5.5 (p-value = 0.015, OR = 4.22, CI: 1.32-13.50) were significantly associated with moderate and severe PE. In a parallel matter, at the time of echocardiography hypoalbuminemia (p-value = 0.004, OR = 5.38, CI: 1.74-16.65) and neutrophilia (p-value = 0.005, OR = 7.94, CI: 1.89-33.44) were significantly associated with moderate and severe PE. CONCLUSION: Despite advancements in the diagnosis and treatment of CKD, PE is still a concerning issue in these patients. This study revealed that hypoalbuminemia, neutrophilia, and NLR greater than 5.5 could be predictive factors of moderate and severe PE in CKD patients with PE. Further prospective study with larger sample size is needed to confirm these results.


Subject(s)
Pericardial Effusion , Renal Insufficiency, Chronic , Humans , Female , Male , Renal Insufficiency, Chronic/complications , Pericardial Effusion/complications , Cross-Sectional Studies , Middle Aged , Aged , Severity of Illness Index , Neutrophils/pathology , Echocardiography
5.
Sultan Qaboos Univ Med J ; 24(2): 283-287, 2024 May.
Article in English | MEDLINE | ID: mdl-38828243

ABSTRACT

Restrictive cardiomyopathy is one of the rarest forms of cardiomyopathies in paediatric patients characterised by impaired myocardial relaxation or compliance with restricted ventricular filling, leading to a reduced diastolic volume with a preserved systolic function. We report 2 cases-a 5-year-old boy who presented with abdominal distension and palpitation with family history of similar complaints but no definite genetic diagnosis as yet and a 5-year-old girl who presented with chronic cough and shortness of breath. Both cases were diagnosed in a tertiary care hospital in Muscat, Oman, in 2019 and are managed supportively with regular outpatient follow-up. This is the first series of reported cases of paediatric restrictive cardiomyopathy from Oman.


Subject(s)
Cardiomyopathy, Restrictive , Humans , Cardiomyopathy, Restrictive/diagnosis , Child, Preschool , Male , Female , Oman , Echocardiography/methods
7.
J Cardiovasc Med (Hagerstown) ; 25(7): 529-538, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38829939

ABSTRACT

AIMS: This study assessed the outcomes of concomitant mitral valve disease and severe aortic stenosis in patients undergoing transcatheter aortic valve replacement (TAVR). METHODS: Echocardiographic data of 813 patients with severe aortic stenosis undergoing transfemoral TAVR were collected, and clinical outcomes were analyzed for individuals with mitral stenosis and mitral regurgitation. RESULTS: The final cohort includes 788 patients with severe calcific aortic stenosis. Among single parameters of mitral stenosis, a smaller baseline mitral valve area (MVA) by the continuity equation and higher postprocedural mean mitral gradients (MMG) were associated with an increased risk of death at 1 year (P-values 0.02 and <0.01, respectively), but no correlation with outcomes was demonstrated after multivariate adjustment for major prognosticators. Mitral stenosis (based on MVA + MMG) was not associated with complications or mortality. Mitral regurgitation was present in 94.6% of the population at baseline and regressed by at least one grade post-TAVR in 28% of the patients. The improvement in mitral regurgitation was associated with a greater prosthetic effective orifice area (P-value 0.03). Significant (at least moderate) residual mitral regurgitation was correlated with short-term complications and shown to be an independent predictor of 1-year mortality (P-value 0.02, odds ratio (OR) 5.37, confidence interval 1.34-21.5). CONCLUSION: Mitral regurgitation has a greater impact on TAVR patients than mitral stenosis as assessed by functional methods.


Subject(s)
Aortic Valve Stenosis , Mitral Valve Insufficiency , Mitral Valve Stenosis , Transcatheter Aortic Valve Replacement , Humans , Male , Female , Mitral Valve Stenosis/surgery , Mitral Valve Stenosis/diagnostic imaging , Mitral Valve Stenosis/mortality , Mitral Valve Stenosis/physiopathology , Mitral Valve Stenosis/complications , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/physiopathology , Mitral Valve Insufficiency/mortality , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/complications , Transcatheter Aortic Valve Replacement/adverse effects , Transcatheter Aortic Valve Replacement/mortality , Aged, 80 and over , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/mortality , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/physiopathology , Aortic Valve Stenosis/complications , Aged , Treatment Outcome , Severity of Illness Index , Retrospective Studies , Prognosis , Risk Factors , Aortic Valve/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Aortic Valve/pathology , Calcinosis/mortality , Calcinosis/diagnostic imaging , Calcinosis/complications , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Mitral Valve/physiopathology , Echocardiography
8.
Front Endocrinol (Lausanne) ; 15: 1384514, 2024.
Article in English | MEDLINE | ID: mdl-38836221

ABSTRACT

Introduction: Type 1 diabetes (T1D) is a metabolic disease characterized by insulin deficiency and subsequent hyperglycemia. Cardiovascular diseases are the prime cause of mortality and morbidity among patients with T1D. Accumulating metabolic disturbances and accelerated cardiac fibrosis fuel the development of heart dysfunction. As insulin resistance (IR) is a risk factor for the development and worsened course of heart failure, this study aimed to assess its impact on heart function in patients with T1D. Methods: Adult participants were recruited prospectively. The inclusion criteria included a diagnosis of T1D. The exclusion criteria were other types of diabetes, symptoms/treatment of heart failure, AST and/or ALT exceeding the upper reference limit by ≥2x, hepatitis, alcoholism, metformin treatment, and pregnancy. The participants underwent a medical interview, physical examination, biochemical test, and echocardiography. Results: The mean age in the study group was 38 ± 9.6 years, and the mean diabetes duration was 21.8 ± 11.3 years. The median BMI in the study cohort was 23.39 kg/m2. Patients with IR had significantly lower mitral E/A ratio and left ventricular and left atrial volume ratio (LVLAVR), higher LV mass index, and presented with altered mitral annular velocities. Conclusions: IR seems to accelerate the pattern of typical changes in heart function among patients with T1D, especially in the overweight subgroup.


Subject(s)
Diabetes Mellitus, Type 1 , Insulin Resistance , Overweight , Humans , Female , Male , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/physiopathology , Adult , Overweight/complications , Overweight/physiopathology , Middle Aged , Prospective Studies , Echocardiography
9.
Heart Fail Clin ; 20(3): 271-282, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38844298

ABSTRACT

Amyloidosis is a rare, heterogeneous group of diseases characterized by extracellular infiltration and deposition of misfolded fibrils in different organs and tissues. A timely diagnosis is important as it can improve outcome. Echocardiography has emerged as a powerful tool to prompt suspicion and refer patients to second-level evaluation to reach a definitive diagnosis. In this scenario, new echo techniques offer new insight into the cardiac amyloidosis (CA) pathophysiology and clinical course. The present review aims to describe the developments in echocardiographic assessment of patients with suspected CA and it summarizes new available echocardiographic scores able to guide a definite diagnosis.


Subject(s)
Amyloidosis , Cardiomyopathies , Echocardiography , Humans , Amyloidosis/diagnostic imaging , Amyloidosis/therapy , Amyloidosis/diagnosis , Echocardiography/methods , Cardiomyopathies/diagnostic imaging , Cardiomyopathies/therapy , Risk Assessment , Disease Management
10.
Echocardiography ; 41(6): e15851, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38837500

ABSTRACT

Loeys-Dietz syndrome (LDS) is an autosomal-dominant connective tissue disorder associated with mutations in the transforming growth factor ß receptor. It is characterized by distinctive craniofacial changes, skeletal features, and cardiovascular complications. We present a case of a 24-year-old male with development delay and a one-year history of progressively worsening dyspnea on moderate exertion and orthopnea. Echocardiography revealed right atrial and right ventricle dilation, right ventricle hypertrophy, atrial septal defect, and aneurysmal dilation of the pulmonary artery trunk. This case underscores the importance of early detection and comprehensive imaging in patients suspected of having LDS, particularly considering the potential for atypical vascular manifestations.


Subject(s)
Delayed Diagnosis , Echocardiography , Heart Septal Defects, Atrial , Loeys-Dietz Syndrome , Pulmonary Artery , Humans , Male , Loeys-Dietz Syndrome/complications , Loeys-Dietz Syndrome/diagnosis , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/diagnosis , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/abnormalities , Young Adult , Echocardiography/methods , Dilatation, Pathologic , Diagnosis, Differential
11.
Echocardiography ; 41(6): e15852, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38837738

ABSTRACT

PURPOSE: To assess left atrial (LA) function in individuals with known paroxysmal atrial fibrillation (AF) compared with healthy and nonhealthy individuals without atrial fibrillation. METHODS: The Akershus Cardiac Examination 1950 Study included 3,706 individuals all born in 1950. LA strain assessment of reservoir (LASr), conduit (LAScd) and contractile (LASct) functions were performed in all participants by investigators blinded to clinical data. Participants with cardiovascular disease, obesity, diabetes, pulmonary or renal disease were defined as nonhealthy, and those without as healthy. Patients with paroxysmal AF were identified through medical history and ECG documentation. RESULTS: LA strain assessment was feasible in 3,229 (87%) of the participants (50% women). The healthy group (n = 758) had significantly higher LASr and LAScd than the nonhealthy (n = 2,376), but LASct was similar between the groups. Participants with paroxysmal AF had significantly lower values of all strain parameters than the other groups. Multivariable logistic regression showed a significantly reduced probability of having AF per standard deviation increase in LASr and LASct. A nonlinear restricted cubic spline model fitted better with the association of LASr with paroxysmal AF than the linear model, and LA strain values below the population mean associated with an increased probability of having AF, but for values above the population mean no such association was present. CONCLUSION: Compared to participants without AF, those with known paroxysmal AF had significantly lower values of all LA strain parameters during sinus rhythm. Lower values of LA strain were associated with a significantly increased probability of having AF.


Subject(s)
Atrial Fibrillation , Heart Atria , Humans , Atrial Fibrillation/physiopathology , Atrial Fibrillation/diagnosis , Female , Male , Middle Aged , Heart Atria/physiopathology , Heart Atria/diagnostic imaging , Atrial Function, Left/physiology , Echocardiography/methods
12.
Interv Cardiol Clin ; 13(3): 355-368, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38839169

ABSTRACT

Transcatheter ductal closure has proven to be safe and effective to occlude the patent ductus arteriosus (PDA) in premature infants. We have developed a robust multidisciplinary PDA program. After gaining experience performing transcatheter ductal closure in the cardiac catheterization laboratory, we transitioned to performing the procedure at the patient's bedside in the neonatal intensive care unit using echocardiographic guidance. The bedside approach has the potential to expand this therapy to patients who currently undergo expectant PDA management because of lack of availability of this therapy, and allow for earlier referral, which will reduce time of exposure to this pathologic shunt.


Subject(s)
Cardiac Catheterization , Ductus Arteriosus, Patent , Infant, Premature , Humans , Ductus Arteriosus, Patent/surgery , Ductus Arteriosus, Patent/diagnosis , Infant, Newborn , Cardiac Catheterization/methods , Septal Occluder Device , Echocardiography/methods
15.
Minerva Pediatr (Torino) ; 76(3): 321-327, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38842379

ABSTRACT

BACKGROUND: Left ventricular hypertrophy (LVH) is an important complication of infants of diabetic mothers (IDMs). However, the defined factors, such as the influence of glycemic control, insulin administration of diabetic mothers and large for gestational age (LGA) in infants, are largely unknown on the incidence of LVH. Therefore, this study aimed to evaluate the prevalence of maternal and neonatal risk factors associated with LVH in IDMs. METHODS: This prospective analytic study was conducted at tertiary care hospitals in a 1-year period. Inborn IDMs were enrolled, and ventricular hypertrophy was identified by 2D echocardiography in the first 72 hours after birth. RESULTS: A total of 160 IDMs met the inclusion criteria, 33 (20.6%) of which had LVH. The incidence of infants with LVH born to mothers with poor glycemic control (fasting blood sugar >95 mg/dL) was significantly elevated than those with good glycemic control (45.5% vs. 14.4%, P<0.001). Twelve IDMs (12/33, 36.5%) of LVH and 17 IDMs (17/127, 13.4%) of non-LVH were LGA. IDMs with LVH, compared those with non-LVH, had significantly increased left ventricular (LV) geometry; IVSd (6.5±0.8 vs. 4.0±0, 7 mm), LV IDd (16.8±3.3 mm vs. 18.4±1.1), left ventricular ejection fraction (LVEF) (68.3±8.5% vs. 62.9±17.5%), left ventricular fraction shortening (LVFS) (35.9±6.6% vs. 32.2±5.5%), LV mass (15.3±11.6 vs. 9.3±2.5 g) and LV mass index (66.2±17.5 vs. 46.6±9.7 g/m2), all with P<0.001. There was significant correlation in LV mass with infants' weight, height and body surface area (BSA) (r=0.408, 0.337 and 0.424, respectively; P<0.001). CONCLUSIONS: The prevalence of neonatal ventricular hypertrophy in IDMs was 20.6%. Maternal poor glycemic control and LGA status in IDMs were dominant risk factors of LVH.


Subject(s)
Echocardiography , Glycemic Control , Hypertrophy, Left Ventricular , Humans , Hypertrophy, Left Ventricular/epidemiology , Hypertrophy, Left Ventricular/etiology , Female , Infant, Newborn , Prospective Studies , Pregnancy , Risk Factors , Male , Adult , Pregnancy in Diabetics/epidemiology , Incidence , Prevalence , Insulin/therapeutic use , Blood Glucose/analysis
16.
PLoS One ; 19(6): e0304724, 2024.
Article in English | MEDLINE | ID: mdl-38829873

ABSTRACT

The effects of conditioning on cardiac function in young horses is still unknown. For this reason, this study evaluated the left ventricular (LV) function of young horses by echocardiography after six weeks of conditioning. Fourteen untrained young purebred Arabian horses were evaluated at rest and after a stress test (ST) before and after a six-week conditioning program. There was an increase in V4 (p < 0.001) after conditioning, as well as a reduction in both heart rate (HR) at rest and peak HR during the ST (p < 0.001). There was also a reduction in internal diameter, along with an increase in interventricular septal, free wall and mean thicknesses and LV mass (p < 0.05). After the ST, the conditioned animals showed higher values of velocity time integral, stroke volume, systolic and cardiac indices, ejection (ET) and deceleration times (DT), end-diastolic volume, time to onset of radial myocardial velocity during early diastole and time to peak of transmitral flow velocity, in addition to reduced pre-ejection period (PEP), PEP/ET ratio and mean velocity of circumferential fiber shortening (p < 0.05). The conditioning protocol promoted physiological adaptations that indicate an improvement in the animals' aerobic capacity associated with an enhanced left ventricular function.


Subject(s)
Echocardiography , Heart Rate , Physical Conditioning, Animal , Ventricular Function, Left , Animals , Horses/physiology , Ventricular Function, Left/physiology , Physical Conditioning, Animal/physiology , Heart Rate/physiology , Male , Female , Stroke Volume/physiology
17.
Saudi Med J ; 45(6): 572-577, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38830663

ABSTRACT

OBJECTIVES: To evaluate the relationship between severity of tricuspid regurgitation (TR) and pulmonary hypertension. METHODS: Cross-sectional study of 118 patients with pulmonary hypertension was carried out at a single center in Jeddah, Saudi Arabia, between 2018-2021. Patients who had pulmonary or tricuspid valves organic diseases, previously undergone tricuspid or pulmonary valve surgeries, had permanent pacemakers or critically ill were excluded. RESULTS: A high proportion of patients were women (n=100, 85%) and obese (n=57, 48%). Patients with more than mild TR had higher systolic pulmonary artery pressure (sPAP) than those with trivial or mild regurgitation (p<0.001). There was a significant association between severity of TR (p<0.001) and right chambers size (p=0.001). Furthermore, pulmonary artery pressure (PAP) was significantly higher in patients with mild right ventricular impairment (p=0.001). CONCLUSION: Increase in degree of TR and right atrial size were predictors of elevated sPAP. Our findings highlight the interplay among TR, right heart size, ventricular function, and PAP. Understanding these associations can aid in risk stratification, monitoring disease progression, and potentially guiding treatment in those patients.


Subject(s)
Hypertension, Pulmonary , Severity of Illness Index , Tricuspid Valve Insufficiency , Humans , Tricuspid Valve Insufficiency/physiopathology , Female , Male , Hypertension, Pulmonary/physiopathology , Cross-Sectional Studies , Middle Aged , Adult , Saudi Arabia/epidemiology , Ventricular Dysfunction, Right/physiopathology , Aged , Heart Atria/physiopathology , Obesity/complications , Obesity/physiopathology , Echocardiography
18.
Commun Biol ; 7(1): 682, 2024 Jun 14.
Article in English | MEDLINE | ID: mdl-38877299

ABSTRACT

Although the gross morphology of the heart is conserved across mammals, subtle interspecific variations exist in the cardiac phenotype, which may reflect evolutionary divergence among closely-related species. Here, we compare the left ventricle (LV) across all extant members of the Hominidae taxon, using 2D echocardiography, to gain insight into the evolution of the human heart. We present compelling evidence that the human LV has diverged away from a more trabeculated phenotype present in all other great apes, towards a ventricular wall with proportionally greater compact myocardium, which was corroborated by post-mortem chimpanzee (Pan troglodytes) hearts. Speckle-tracking echocardiographic analyses identified a negative curvilinear relationship between the degree of trabeculation and LV systolic twist, revealing lower rotational mechanics in the trabeculated non-human great ape LV. This divergent evolution of the human heart may have facilitated the augmentation of cardiac output to support the metabolic and thermoregulatory demands of the human ecological niche.


Subject(s)
Heart Ventricles , Hominidae , Phenotype , Animals , Humans , Heart Ventricles/anatomy & histology , Heart Ventricles/diagnostic imaging , Hominidae/anatomy & histology , Echocardiography , Biological Evolution , Pan troglodytes/anatomy & histology , Male , Female
19.
BMJ Case Rep ; 17(6)2024 Jun 12.
Article in English | MEDLINE | ID: mdl-38866580

ABSTRACT

Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.


Subject(s)
Hydrops Fetalis , Mitral Valve Insufficiency , Mitral Valve , Humans , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/surgery , Hydrops Fetalis/therapy , Hydrops Fetalis/diagnostic imaging , Male , Infant, Newborn , Mitral Valve/abnormalities , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Echocardiography , Heart Failure/etiology , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/therapy , Positive-Pressure Respiration/methods
20.
J Investig Med High Impact Case Rep ; 12: 23247096241248969, 2024.
Article in English | MEDLINE | ID: mdl-38869103

ABSTRACT

Constrictive pericarditis (CP) presents as a pathophysiological state where the pericardium becomes inelastic due to fibrotic changes, most commonly secondary to a protracted inflammatory process. The disease is characterized by compromised diastolic cardiac function due to loss of pericardial compliance. Immunoglobulin G4 (IgG4)-related disease, an entity marked by the insidious proliferation of IgG4-positive plasma cells and subsequent fibrosis within various organs, is an infrequent but recognized cause of CP. A case of a 55-year-old male patient with clinical manifestations of dyspnea and edema in the lower extremities elucidates the diagnostic complexity inherent to CP. Echocardiography revealed a constellation of signs, including annulus reversus, septal bounce, and a congested inferior vena cava; cardiac magnetic resonance imaging (MRI) demonstrated diffuse pericardial thickening with delayed gadolinium enhancement, suggestive of a long-term inflammatory state; and right heart catheterization confirmed the hemodynamic hallmark of CP-equalization of diastolic pressures across the cardiac chambers. The serological analysis elicited elevated serum levels of IgG4 and IgE, pointing to the differential diagnosis of IgG4-related disease. Given the nonspecific clinical presentation of IgG4-related CP, a heightened index of suspicion combined with a systematic approach to imaging and serological evaluation is paramount.


Subject(s)
Echocardiography , Immunoglobulin G , Magnetic Resonance Imaging , Multimodal Imaging , Pericarditis, Constrictive , Humans , Pericarditis, Constrictive/diagnostic imaging , Pericarditis, Constrictive/diagnosis , Male , Middle Aged , Immunoglobulin G/blood , Immunoglobulin G4-Related Disease/diagnosis , Immunoglobulin G4-Related Disease/diagnostic imaging , Immunoglobulin G4-Related Disease/complications , Diagnosis, Differential
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