-
JACC. Case Reports Apr 2024This clinical vignette describes the first case of a woman in his 40s with a set of congenital anomalies given by tetralogy of Fallot associated with aortic origin of...
This clinical vignette describes the first case of a woman in his 40s with a set of congenital anomalies given by tetralogy of Fallot associated with aortic origin of the left pulmonary artery and uncorrected right aortic arch. All of these entities have a poor probability of survival in adulthood.
PubMed: 38774807
DOI: 10.1016/j.jaccas.2024.102277 -
Cureus Mar 2024This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various...
This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various cardiovascular complexities associated with this syndrome. While current treatments focus on managing symptoms, ongoing genetic research offers hope for more favorable outcomes. Further studies are crucial to gauge the effectiveness of these treatments for PWS patients. We detail a patient with a complex medical history of PWS, further complicated by congenital heart disease with Eisenmenger's syndrome, diabetes mellitus, pulmonary hypertension, venous insufficiency, hypothyroidism, and hyperlipidemia. Reported in this study is a compilation of clinical data as well as suggestions from several medical specialists in applying a multifaceted approach to treatment, significantly emphasizing the need for interdisciplinary care and management of patients experiencing a combination of various medical issues with an emphasis on cardiovascular complications.
PubMed: 38646247
DOI: 10.7759/cureus.56591 -
Journal of the American Heart... May 2024Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However,...
BACKGROUND
Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However, the risk stratification for persistent pulmonary hypertension (PH) in this population remains uncertain.
METHODS AND RESULTS
We retrospectively enrolled 103 patients with pretricuspid shunts with high total pulmonary resistance >4.5 Wood units (estimated pulmonary vascular resistance ≥3 Wood units). During a mean±SD follow-up of 20.95±24.84 months, 32 patients developed postoperative persistent PH after shunt correction. We identified 3 significant predictors of postoperative persistent PH, including mean pulmonary artery pressure after inhaled oxygen ≥40.5 mm Hg (odds ratio [OR], 7.78 [95% CI, 2.02-30.03]; <0.01), total pulmonary resistance after inhaled oxygen ≥6.5 Wood units (estimated pulmonary vascular resistance ≥5 Wood units; OR, 12.23 [95% CI, 2.12-70.46]; <0.01), and artery oxygen saturation at rest <95% (OR, 3.34 [95% CI, 1.07-10.44]; =0.04). We established the prediction model with the C-statistics of 0.85 (95% CI, 0.77-0.93; <0.01), and the C-statistic was 0.83 (95% CI, 0.80-0.86) after bootstrapping 10 000 times with a good performance of the nomogram calibration curve for predicting persistent PH.
CONCLUSIONS
Our study presents a multivariable risk stratification model for persistent PH after shunt correction in adults with pretricuspid shunts. This model, based on 3 hemodynamic predictors after inhaled oxygen, may assist in identifying individuals at higher risk of persistent PH after shunt correction.
Topics: Humans; Female; Male; Hypertension, Pulmonary; Retrospective Studies; Nomograms; Adult; Vascular Resistance; Risk Assessment; Pulmonary Artery; Middle Aged; Risk Factors; Predictive Value of Tests; Treatment Outcome; Cardiac Surgical Procedures; Heart Defects, Congenital; Arterial Pressure
PubMed: 38639332
DOI: 10.1161/JAHA.123.032412 -
Cureus Feb 2024Eisenmenger syndrome (ES) is a complex, multisystemic, and rare clinical entity, given that currently, most congenital heart diseases can be corrected in childhood. The...
Eisenmenger syndrome (ES) is a complex, multisystemic, and rare clinical entity, given that currently, most congenital heart diseases can be corrected in childhood. The high anesthetic risk in these patients poses a challenge for anesthesiology. There are few cases described in the literature of anesthetic approaches using ketamine and dexmedetomidine in ES cases, particularly under Monitored Anesthesia Care (MAC). We describe the clinical case of a 40-year-old patient with trisomy 21, intellectual disability, and ES secondary to a single atrioventricular (AV) valve, scheduled for cranial magnetic resonance imaging (MRI) under sedation due to a suspected space-occupying lesion. Sedation was performed under MAC with dexmedetomidine and ketamine. The procedure proceeded without complications. The anesthetic approach in ES patients, given the clinical complexity, requires planning by a multidisciplinary team and should be tailored to the procedure and its duration.
PubMed: 38500910
DOI: 10.7759/cureus.54285 -
Kidney & Blood Pressure Research 2024Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may... (Review)
Review
INTRODUCTION
Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES.
CASE PRESENTATION
A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength.
CONCLUSION
Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.
Topics: Humans; Female; Adult; Eisenmenger Complex; Kidney Diseases; Cyanosis; Polycythemia
PubMed: 38447536
DOI: 10.1159/000538100 -
Medicina (Kaunas, Lithuania) Feb 2024Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with...
Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently. A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24-48 h of admission. This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD.
Topics: Adult; Male; Humans; Middle Aged; Hypertension, Pulmonary; Cardiovascular Diseases; Heart Defects, Congenital; Eisenmenger Complex; Heart Failure
PubMed: 38399553
DOI: 10.3390/medicina60020266 -
Cureus Jan 2024Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly...
Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly improved long-term outcomes, some individuals, particularly those from low socioeconomic backgrounds who lack access to medical care, may suffer from complications such as pulmonary hypertension (pHTN) and heart failure. We present a case report of a young female with unrepaired TOF who presented with acute-on-chronic hypoxic respiratory failure and heart failure, highlighting the complex nature and challenges associated with this condition.
PubMed: 38371077
DOI: 10.7759/cureus.52407 -
Frontiers in Cardiovascular Medicine 2024A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to...
Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.
AIMS
A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA).
METHODS AND RESULTS
Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed. Patients were classified according to predefined ABCDE categories (A: Eisenmenger syndrome, B: left-to-right shunt, C: coincidental defects, including all ASDs, D: corrected CHD, E: TGA), or as complex CHD (group 5), by 2 independent investigators. In case of disagreement, a third reviewer could either settle a final decision, or the patient was deemed not classifiable. Survival curves were calculated for each group and compared to idiopathic PAH patients of the registry. A total of 223 out of 531 patients in the registry had PAH-CHD, and 193 were categorized to the following groups: A 39(20%), B 27(14%), C 62(32%) including 43 ASDs, D 58(30%), E 7(4%), whereas 6 patients were categorized as group 5, and 10 patients were unable to be classified. No survival difference could be demonstrated between the groups.
CONCLUSIONS
This modified classification seems to be more applicable to pediatric PAH-CHD patients than the previous classification, but some patients with PAH-CHD who never had a shunt remain unclassifiable. The role of ASD in pediatric PH should be reconsidered.
PubMed: 38370158
DOI: 10.3389/fcvm.2024.1344014 -
Polish Archives of Internal Medicine Feb 2024
Topics: Humans; Eisenmenger Complex; Hypertension, Pulmonary
PubMed: 38133886
DOI: 10.20452/pamw.16652 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Oct 2023Pulmonary arterial hypertension (PAH) is a profoundly destructive condition marked by the gradual narrowing and restructuring of small pulmonary arteries, leading to a...
Pulmonary arterial hypertension (PAH) is a profoundly destructive condition marked by the gradual narrowing and restructuring of small pulmonary arteries, leading to a rise in pulmonary vascular resistance (PVR), causing right-sided heart failure and, ultimately, mortality. During more advanced stages of this disease, patients may present with rare manifestations of pulmonary artery aneurysm (PAA) which are exertional chest pain, and hoarseness. The left main coronary artery compression (LMCA-Co) has been an increasingly recognized and possibly life-threatening entity in patients with severe PAH. The lack of well-established decision-making strategies for the management of both PAA and LMCA-Co has been remained as an unsolved issue in this setting. In this report, we present a case of percutaneous intervention of LMCA-Co with the guidance of intracoronary imaging in a patient with patent ductus arteriosus-Eisenmenger syndrome. Percutaneous intervention with intravascular guidance appears to be a safe and effective option for relieving symptoms and achieving positive clinical outcomes in patients with LMCA-Co.
Topics: Humans; Eisenmenger Complex; Pulmonary Artery; Coronary Vessels; Coronary Stenosis; Tomography, X-Ray Computed; Coronary Angiography; Angioplasty, Balloon, Coronary; Aneurysm; Stents; Ultrasonography, Interventional
PubMed: 37861262
DOI: 10.5543/tkda.2023.56585