scholarly journals Large Aorto-Pulmonary Window (APW) associated with severe pulmonary hypertension: A case report

2017 ◽  
Vol 1 (1) ◽  
pp. 29
Author(s):  
Abdul Wasay Khan

Background: APW is a rare congenital cardiac abnormality, accounting for 0.2% to 0.6% of patients. Hence the reported surgical experience is minimal. It is a     communication b/w pulmonary artery (PA) and the ascending aorta (Ao), in the presence of two separate semi lunar valves. Heart failure and irreversible pulmonary hypertension are the fearsome outcomes of untreated APW. Therefore, surgical  closure is the first-line curative choice. We present a case of young boy who presented late in age (his second decade of life) with severe signs and symptoms. Case   Presentation: A 12-year-old male patient from Baluchistan (balochi) presented with complaints of high grade fever for one week, palpitations since 12 days, along with past history of shortness of breath. On examination patient was anemic, cyanosed with raised JVP. On precordial examination apex beat was localized at the 6th     intercostal space shifted to mid-axillary line. S1 was normal but S2 was loud with prominent pulmonary component. Grade IV/VI continuous murmur was heard in the left upper sterna border. Chest X-ray showed cardiomegaly with prominent pulmonary artery (PA) and bilateral lung congestion. Echocardiography showed enlarged and hypertrophied right ventricle associated with dilated PA and a large APW was seen with severe pulmonary artery pressure (PAP) of 102 mmHg. Diagnosis of APW was confirmed on cardiac catheterization. Furthermore, cardiac CT was performed which showed type I APW with severe PH and large patent PDA (patent ductus  arteriosus). Patient then underwent repair of APW associated with reactive     pulmonary hypertension that had a complete repair utilizing a trans-aortic approach. PDA was also ligated during the same procedure. Patient symptoms remarkably  improved with a reduction PAP (40mmHg) after surgery and at 6 weeks follow-up. Follow-up echocardiography showed complete closure of the APW, and       pharmacological therapy was no longer required in the patient. Conclusion: APW should be suspected in any child, who comes with repeated chest infections and signs of congestive heart failure. It should be surgically corrected early, in order to prevent the development of obstructive pulmonary vascular disease. There is conflict of   interest.

1996 ◽  
Vol 4 (3) ◽  
pp. 164-167
Author(s):  
Binali Mavitaş ◽  
S Fehmi Katircioğlu ◽  
Birol Yamak ◽  
Ahmet Saritaş ◽  
Gürkan Uzunonat ◽  
...  

Between 1968 and 1995, a total of 153 patients who were between 35 and 63 years of age (mean 49.8 years), underwent surgery for secundum type atrial septal defect. There were 78 (50.9%) males and 75 females (49.1 %). Mean left-to-right shunt ratio was calculated as 2.49. Mean pulmonary artery pressure was 50.15 mm Hg. Three patients died within 30 days of surgery, giving a hospital mortality of 1.96%. Long-term follow-up was available in 135 cases (90%). Total follow-up was 967.3 patient-years and ranged from 3 months to 11.3 years (mean 7.16 years). There were no late deaths reported. Four patients were readmitted with atrial fibrillation and 2 with pericardial effusion. In our experience, surgical closure of atrial septal defect in adults was found to be successful, safe, and with low morbidity in patients with pulmonary hypertension and congestive heart failure.


2020 ◽  
Vol 30 (6) ◽  
Author(s):  
Henok Tadele ◽  
Etsegenet Gedlu

BACKGROUND፡ Unilateral pulmonary artery absence or discontinuity is a rare congenital abnormality. Its reported prevalence is 1 in 150,000 adults.CASE PRESENTATION: A 22-month-toddler girl was referred from a general hospital for cardiac follow-up to our hospital after diagnosis of severe pulmonary hypertension was made. She presented with generalized body swelling, cough, fast breathing and high-grade fever of four days duration. She had repeated severe chest infections. Echocardiography and chest computed tomography revealed right pulmonary artery discontinuity. Acute care was given and chronic care was established.CONCLUSION:Pulmonary artery discontinuity is a rare anomaly. It should be considered in any child presenting with recurrent chest infections/pulmonary hypertension. Appropriate and timely diagnostic work up will avoid misdiagnosis.


2018 ◽  
Vol 9 (2) ◽  
pp. 204589401881629 ◽  
Author(s):  
Hang Zhang ◽  
Wande Yu ◽  
Juan Zhang ◽  
Dujiang Xie ◽  
Yue Gu ◽  
...  

This study aimed to determine the benefits and correlated mechanisms of pulmonary artery denervation (PADN) for heart failure (HF) pulmonary hypertension (PH). PH secondary to HF is associated with poor clinical outcomes because there is no proper therapy for it. PADN showed improved outcomes for patients with HF-PH. However, the underlying mechanisms remain unknown. Supracoronary aortic banding (SAB) was used to create HF-PH models. Sprague-Dawley rats were randomly assigned to control, SAB, sham, SAB with PADN, and SAB without PADN groups. Surgical (longitudinally damaging vessel nerves) and chemical (10% phenol applied to the surface of nerves) PADN was performed for animals in the SAB with PADN group. Morphological, echocardiographic, hemodynamic, and protein expression changes were measured four weeks thereafter. Adrenergic receptor (AR) expressions of pulmonary arteries from four HF-PH patients and four patients without PH were measured. Ten HF-PH patients who underwent PADN were followed-up for six months. SAB-induced HF-PH was achieved by 50% of animals. Surgical and chemical PADN was associated with significant improvements in pulmonary artery muscularization, hemodynamics, and right ventricular functions. In pulmonary arterial specimens from HF-PH patients, β2-AR and α1A/B-AR, as well as eNOS, were downregulated and α1D-AR was upregulated compared to those from patients without PH. PADN led to a mean increase of 84 m during the 6-min walk distance for HF-PH patients at six-month follow-up. HF-PH was characterized by downregulated β2-AR, α1A-AR, and α1B-AR and by upregulated α1D-AR. PADN is associated with significant improvements in hemodynamics and pulmonary artery remodeling.


2020 ◽  
Vol 30 (12) ◽  
pp. 1943-1945
Author(s):  
Semih Murat Yucel ◽  
Irfan Oguz Sahin

AbstractDuctus arteriosus is an essential component of fetal circulation. Due to occurring changes in the cardiopulmonary system physiology after birth, ductus arteriosus closes. Patent ductus arteriosus can be closed by medical or invasive (percutaneous or surgical) treatment methods. Percutaneous or surgical closure of patent ductus arteriosus can be performed for the cases that medical closure failed. Surgical treatment is often preferred method for closure of patent ductus arteriosus in the neonatal period. The most common surgical complications are pneumothorax, recurrent laryngeal nerve injury, bleeding, and recanalisation. A very rare surgical complication is left pulmonary artery ligation that has been presented in a few cases in the literature. Echocardiography control should be performed in the early post-operative period, especially in patients with clinical suspicion. If reoperation is required, it should never be delayed. We report a newborn patient whose left pulmonary artery ligated accidentally during patent ductus arteriosus closure surgery and surgical correction of this complication at the early post-operative period.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Jeong-Hoon Kim ◽  
Duk-Hyun Kang ◽  
Jong-Young Lee ◽  
Jong-Min Song ◽  
Tae-Jin Yun ◽  
...  

The benefits of surgical closure has been unclear in adult atrial septal defect (ASD) with severe pulmonary hypertension (PHT), and we tried to evaluate improvement of PHT and long-term survival after surgical closure compared to medical follow-up. Methods: From 1996 to 2006, we included a total of 71 adult ASD patients (age; 43±15 years) with severe PHT documented by echocardiography. The inclusion criteria were defined as ASD diameter > 15 mm, enlarged right ventricle, and the baseline peak velocity of tricuspid regurgitation (TR) ≥ 4.0 m/sec. We excluded 5 patients with Eisenmenger syndrome documented by cardiac catheterization. Surgical closure was performed on 55 patients (OP group) and the remaining 16 patients were followed up medically (MED group). The improvement of PHT was defined as TR velocity ≤ 3.5 m/sec on follow-up echo. Results: Baseline characteristics and clinical results were compared between the two groups in table . There were no significant differences in terms of gender, ASD diameter, cardiac rhythm, and TR velocity, but the MED group was significantly older. During follow-up of 46±33 months, there were 5 deaths in the MED group and no operative or late death in the OP group, and the 5-year actuarial survival rate of the OP group was significantly higher than the MED group (58±15%, p<0.05). On subgroup analysis according to age, the OP group showed significantly better survival rates than the MED group (p<0.05) in elderly patients (age > 50). In the OP group, TR velocity was significantly decreased from 4.5±0.4 to 3.0±0.7 m/sec on follow-up echo, and improvement of PHT was observed in 47 (85%) patients. On multivariate analysis, female gender and lower baseline TR velocity were the significant independent predictors of improved PHT after surgery. Conclusions: In adult ASD with severe PHT, surgical closure can be safely performed and improve PHT effectively. Especially in elderly patients, ASD closure is significantly related with the better survival rates.


2021 ◽  
Vol 70 (Suppl-4) ◽  
pp. S701-05
Author(s):  
Khushal Khan Khattak ◽  
Maad Ullah ◽  
Abdul Malik Sheikh ◽  
Asma Kanwal ◽  
Sajid Ali Shah ◽  
...  

Objective: To determine different treatment options in patients of Patent Ductus Arteriosus with pulmonary hypertension beyond neonatal period. Study Design: Descriptive cross sectional study. Place and Duration of Study: This study was carried out in Pediatric Cardiology department of Rawalpindi Institute of Cardiology, from Jan 2017 to Jan 2019. Methodology: Patients having PDA with pulmonary hypertension were included in the study. Treatment options were divided into percutaneous catheter device closure, surgical ligation of patent ductus arteriosus and palliative treatment. Any adverse event during the procedure was documented. Stratification was done in regard to gender and age group. Post stratification chi square test was applied and p-value less than or equal to 0.05 was considered as significant. Results: Total number of patients included in the study were 37. Mean age (years) of patients (Mean ± SD) was 19.21 ± 8.76. Mean ± SD pulmonary artery pressure was 56.43 ± 11.55 mmHg. Percutaneous catheter device closure was successful in 24 (64.9%) patients, in 7 (18.9%) patients primary surgical PDA ligation was done, 3 (8.1%) patients were advised palliative treatment and in 3 (8.1%) patients adverse events occurred during percutaneous device closure and were thus referred for surgical ligation. Patent ductus Arteriosus Occlutech device was used in 18 (48.6%) patients, Occlutech VSD device was used in 7 (18.5%) patients and in 1 (2.7%) patient AGA duct occluder was used. Conclusion: In patients with patent ductus arteriosus and pulmonary artery hypertension, percutaneous catheter device closure is a safe and effective procedure.


Author(s):  
Guiomar Pinheiro ◽  
Ana Margarida Alves ◽  
Isabel Neves ◽  
Teresa Sequeira

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Blair Suter ◽  
Dylan Hall ◽  
Eric S Ebenroth ◽  
Larry W Markham ◽  
Michael Johansen

Introduction: Pulmonary hypertension (PH) has been described following surgical palliation for transposition of the great arteries (D-TGA). While early pulmonary vascular disease is associated with late repair, the increasing prevalence of late PH has been observed following the intra-atrial baffle procedure (ABP), Mustard/Senning. A previous study from our institution identified patients with precapillary PH. More recent studies have described predominately postcapillary PH, suggesting multiple, differing mechanisms for this complication. Hypothesis: While heart failure is a common complication following ABP and is associated with postcapillary PH, we hypothesize that precapillary PH can be identified in a subset of patients. Methods: A retrospective descriptive study was performed at a single institution. Using 6 th World Symposium on Pulmonary Hypertension definitions. PH was defined as a mean pulmonary artery pressure >20mmHg. Etiology of PH was defined as precapillary (≥3 Wood units) versus postcapillary (pulmonary artery wedge pressure >15 mmHg). Results: We reviewed 157 D-TGA patients following ABP, finding 33 patients with PH. Mean age at last evaluation was 36.6 (14-54) years. Current condition: 22 alive, 7 dead, and 4 lost to follow-up. Etiology of PH: 10 precapillary, 8 postcapillary, 8 mixed, 2 borderline PH, and 2 did not undergo catherization. Additionally, 2 patients had pulmonary venous baffle obstruction and 1 had left lung hypoplasia. Of patients with recent imaging, 21 of 25 had systemic RV systolic dysfunction on echocardiogram or MRI. Conclusions: Late-onset PH in D-TGA following ABP is a significant long-term complication and warrants vigilant surveillance. This highlights the limitations of imaging and need for catheterization for complete evaluation. In this study, we observed precapillary PH in a majority of patients with PH, which differs from some previous studies. Identifying the etiology of PH could drastically alter treatment strategies and has implications for transplant consideration. Further studies are needed to identify the clinical attributes that contribute to this process.


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