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Indian Pacing and Electrophysiology... 202231 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome,...
31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Transcatheter leadless pacemaker was deployed in her RV septum despite some unforeseen technical problems. This patient had intrahepatic interruption of IVC with Azygous continuation draining into SVC but this altered venovascular course was detected only fluoroscopically midway during the pacemaker implantation procedure and this was not detected in the preprocedural transthoracic echocardiography. This abnormal venous course was clearly demonstrated in the cardiac CT which was performed only after completion of the pacemaker implantation procedure in this patient. The technical challenges encountered mainly were mostly during the manipulation of the 27F delivery catheter of Micra through this altered cardiovascular anatomy via transfemoral approach and also due to the presence of septal defects. Thus, transcatheter leadless permanent pacemaker was implanted successfully through transfemoral access in this complex congenital heart disease with interrupted IVC and azygous continuation. Besides transthoracic echocardiography, it may be better to perform transesophageal echocardiography or even preferably radiological imaging like cardiac CT or MRI prior to transcatheter leadless pacemaker implantation in patients with complex congenital heart disease to understand the cardiovascular anatomy and plan the procedure.
PubMed: 35364233
DOI: 10.1016/j.ipej.2022.03.003 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Sep 2014
Topics: Eisenmenger Complex; Female; Humans; Hypertension, Pulmonary
PubMed: 25362944
DOI: 10.5543/tkda.2014.37539 -
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 -
The Journal of Thoracic and... Apr 1994From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight...
From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.
Topics: Adolescent; Adult; Cardiopulmonary Bypass; Cause of Death; Child; Child, Preschool; Follow-Up Studies; Graft Rejection; Heart Arrest, Induced; Heart-Lung Transplantation; Humans; Infant; Lung Transplantation; Missouri; Reoperation
PubMed: 8159051
DOI: No ID Found -
Transplant International : Official... Jul 2019Transplant type for end-stage pulmonary vascular disease remains debatable. We compared recipient outcome after heart-lung (HLT) versus double-lung (DLT)... (Comparative Study)
Comparative Study
Transplant type for end-stage pulmonary vascular disease remains debatable. We compared recipient outcome after heart-lung (HLT) versus double-lung (DLT) transplantation. Single-center analysis (38 HLT-30 DLT; 1991-2014) for different causes of precapillary pulmonary hypertension (PH): idiopathic (22); heritable (two); drug-induced (nine); hepato-portal (one); connective tissue disease (four); congenital heart disease (CHD) (24); chronic thromboembolic PH (six). HLT decreased from 91.7% [1991-1995] to 21.4% [2010-2014]. Re-intervention for bleeding was higher after HLT; (P = 0.06) while primary graft dysfunction grades 2 and 3 occurred more after DLT; (P < 0.0001). Graft survival at 90 days, 1, 5, 10, and 15 years was 93%, 83%, 70%, 47%, and 35% for DLT vs. 82%, 74%, 61%, 48%, and 30% for HLT, respectively (log-rank P = 0.89). Graft survival improved over time: 100%, 93%, 87%, 72%, and 72% in [2010-2014] vs. 75%, 58%, 42%, 33%, and 33% in [1991-1995], respectively; P = 0.03. No difference in chronic lung allograft dysfunction (CLAD)-free survival was observed: 80% & 28% for DLT vs. 75% & 28% for HLT after 5 and 10 years, respectively; P = 0.49. Primary graft dysfunction in PH patients was lower after HLT compared to DLT. Nonetheless, overall graft and CLAD-free survival were comparable and improved over time with growing experience. DLT remains our preferred procedure for all forms of precapillary PH, except in patients with complex CHD.
Topics: Adolescent; Adult; Child; Child, Preschool; Connective Tissue Diseases; Disease-Free Survival; Female; Graft Survival; Heart Defects, Congenital; Heart-Lung Transplantation; Hospital Mortality; Humans; Lung Transplantation; Male; Middle Aged; Postoperative Complications; Preoperative Period; Primary Graft Dysfunction; Pulmonary Arterial Hypertension; Retrospective Studies; Thromboembolism; Young Adult
PubMed: 30735591
DOI: 10.1111/tri.13409 -
Indian Heart Journal 2021Pulmonary arterial hypertension (PAH) - a complex and progressive disease that carries significant morbidity and mortality despite optimal medical treatment. Combination... (Randomized Controlled Trial)
Randomized Controlled Trial
A randomized, double-blind, placebo-controlled study to evaluate sildenafil, ambrisentan combination therapy in pulmonary hypertension, particularly of Eisenmenger syndrome.
Pulmonary arterial hypertension (PAH) - a complex and progressive disease that carries significant morbidity and mortality despite optimal medical treatment. Combination therapy for PAH can be more effective than monotherapy. The present randomized trial compared the safety and efficacy of sildenafil ambrisentan combination therapy with sildenafil monotherapy. Twenty-two patients of Eisenmenger syndrome and five patients of idiopathic PAH were randomized to two arms. There was a significant improvement in NYHA functional class and mean pulmonary artery pressure, while an insignificant improving trend was observed for 6-min walk distance and oxygen saturation, following the 12 weeks of combination therapy. An upfront combination therapy was found to be safe and effective in the management of PAH patients.
Topics: Antihypertensive Agents; Double-Blind Method; Drug Therapy, Combination; Eisenmenger Complex; Humans; Hypertension, Pulmonary; Phenylpropionates; Piperazines; Purines; Pyridazines; Sildenafil Citrate; Sulfones; Treatment Outcome
PubMed: 34627582
DOI: 10.1016/j.ihj.2021.07.007 -
Heart (British Cardiac Society) May 2016Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and...
Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance. Evaluation and treatment of PAH-CHD/PPHVD-CHD can be divided into in different topics. First, defining criteria for operability and initiation of advanced therapies preoperatively and postoperatively is an unresolved issue. Second, management of Eisenmenger syndrome is still an important question, with recent evidence on the severity of the disease and a more rapidly progressive course than previously described. Third, the Fontan circulation with no subpulmonary ventricle requires a distinct discussion, definition and classification since even a mild rise in pulmonary vascular resistance may lead to the so-called failing Fontan situation. Patients with CHD and single-ventricle physiology (Fontan/total cavopulmonary anastomosis) require a particularly stepwise and individualised approach. This consensus statement is on the current evidence for the most accurate evaluation and treatment of increased pulmonary artery pressure and resistance, as well as ventricular dysfunction, in children with congenital heart defects, and provides according practical recommendations. To optimise preoperative and postoperative management in patients with PAH-CHD, diagnostic and treatment algorithms are provided.
Topics: Adolescent; Algorithms; Antihypertensive Agents; Cardiac Catheterization; Cardiac Surgical Procedures; Child; Consensus; Disease Management; Ductus Arteriosus, Patent; Eisenmenger Complex; Fontan Procedure; Heart Bypass, Right; Heart Defects, Congenital; Hemodynamics; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Circulation; Vascular Resistance; Vasodilation; Vasodilator Agents; Ventricular Dysfunction
PubMed: 27053697
DOI: 10.1136/heartjnl-2015-308378 -
Australian Dental Journal Mar 2007This report describes the case management of a 32 year old special needs patient with life-threatening odontogenic infection. The combination of schizophrenia, Down and...
BACKGROUND
This report describes the case management of a 32 year old special needs patient with life-threatening odontogenic infection. The combination of schizophrenia, Down and Eisenmenger syndromes presented significant challenges to managing his oral health, particularly within the rural context. In this case, dental treatment was limited to a full dental clearance during a high risk general anaesthesia session.
METHOD
A comprehensive work-up prior to general anaesthesia was an essential aspect of care. This included a full medical history and examination, communication between medical specialists, the dentist and family consultation. The anaesthetic procedure was undertaken using a careful regimen of drugs and monitoring to minimize the impact on his cardiovascular system. Techniques to minimize bleeding from extraction sites were also important.
RESULTS
Three weeks postsurgically the patient was reviewed and his family reported that he was interacting positively with them after years of surliness and conflict. This was attributed to a managed psychotropic medication regimen and improved dental condition, which has led to a sustained improvement in quality of life.
CONCLUSIONS
The management of acute odontogenic infection for special needs patients in the rural setting requires a local interdisciplinary team approach, careful consideration of related pathophysiology and its potential impact on general anaesthesia, and close consultation with family and carers.
Topics: Adult; Anesthesia, Dental; Dental Care for Chronically Ill; Down Syndrome; Eisenmenger Complex; Humans; Male; Periapical Abscess; Rural Population; Schizophrenia
PubMed: 17500166
DOI: 10.1111/j.1834-7819.2007.tb00467.x -
Journal of Cardiothoracic Surgery Jul 2023Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various...
BACKGROUND
Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation.
CASE PRESENTATIONS
A 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful.
CONCLUSIONS
We have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient's background requiring lung transplantation, and in these cases close postoperative follow-up is required.
Topics: Female; Humans; Adult; Eisenmenger Complex; Aneurysm, False; Thoracic Surgical Procedures; Lung Transplantation; Plastic Surgery Procedures
PubMed: 37393254
DOI: 10.1186/s13019-023-02305-2 -
BMC Cardiovascular Disorders Apr 2020Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse...
BACKGROUND
Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). However, surgical correction is not recommended in patients with irreversible PAH. Herein we report our experience about Eisenmenger's syndrome in simple CHD.
CASE PRESENTATION
From January 2017 to November 2018, a total of 8 CHD patients (3 ventricular septal defects (VSD), 2 atrial septal defects (ASD), and 3 patent ductus arteriosus (PDA), median age, 15.5 years [range, 3-18 years]) with PAH were detected by chest X-ray, electrocardiogram, transthoracic echocardiography (TTE), computed tomographic angiography (CTA) and cardiac catheterization. The median defect diameter, pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR) were 16.5 mm (range, 3-30 mm), 75 mmHg (range, 60-86 mmHg), and 16 Woods units (range, 12-19 Woods units), respectively. Here, we report the representative cases of three types of simple CHD with irreversible PAH. The surgical correction was not performed in all patients who had fixed PAH and were referred to medical treatment.
CONCLUSIONS
PAH in CHD can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return. This article highlights the essence of enhancing the level of healthcare and services in Chinese rural areas. Failure to accurately and timely assess PAH will delay effective treatment past optimal treatment time, and even lead to death.
Topics: Adolescent; Arterial Pressure; Child; Computed Tomography Angiography; Early Diagnosis; Echocardiography; Eisenmenger Complex; Female; Humans; Male; Predictive Value of Tests; Prognosis; Pulmonary Arterial Hypertension; Pulmonary Artery
PubMed: 32326907
DOI: 10.1186/s12872-020-01489-y