Pulmonary Vascular Obstructive Disease Research Articles

Giant Partial Endocardial Cushion Defect Challenging Mitral Valve Replacement in Adults: Surgical Approach.

Atrial septal defect (ASD) is one of the most common congenital heart diseases diagnosed in children and adults [ [1] Steele P.M. Fuster V. Cohen M. Ritter D.G. McGoon D. Isolated atrial septal defect with pulmonary vascular obstructive disease – long-term follow-up and prediction of outcome after surgical correction. Circulation. 1987; 76: 1037-1042 Crossref PubMed Scopus (236) Google Scholar ]. Interventional closure devices or surgical patch closure are potential treatment options in haemodynamically relevant ASD. Concomitant severe mitral valve regurgitation is impeded by persisting type I ASD, as a congenital partial endocardial cushion defect (PECD) might be present. Partial endocardial cushion defect is a rare form of congenital heart failure, accounting for about 3% of the overall incidence [ [2] Robinson C.L. Merriman J.E. Kilduff C.J. Partial endocardial cushion defects. Can Med Assoc J. 1963; 88: 1147-1154 Google Scholar ].

AN AORTOPULMONARY TUNNEL AS AN EXTRA-CARDIAC SYSTEMIC TO PULMONARY SHUNT

Aortopulmonary window, one of the rarest congenital heart diseases, causes unrestricted left-to-right shunt and may lead to congestive heart failure, pulmonary vascular obstructive disease and low cardiac output. Echocardiogram and CT angiography are important non-invasive diagnostic tools. Surgical closure is indicated in all patients diagnosed with APW and is usually performed at the time of diagnosis to reduce the risk of developing early pulmonary vascular disease. We report on a term baby girl with variant of aortopulmonary window which manifest as a tunnel on CT angiography.

Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease.

Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular obstructive disease, and genetic variations of vasoactive mediators. Pulmonary input impedance consists of the pulmonary vascular resistance (Rp) and capacitance (Cp). Rp is calculated as the transpulmonary pressure divided by the pulmonary cardiac output, whereas Cp is calculated as the pulmonary stroke volume divided by the pulmonary arterial pulse pressure. The plots of Rp and Cp demonstrate a unique hyperbolic relationship, namely, the resistor–capacitor coupling curve, which represents the pulmonary vascular condition. The product of Rp and Cp is the exponential pressure decay, which refers to the time constant. Alterations in Cp are more considerable in CHD patients at an early stage of developing pulmonary hypertension or with excessive pulmonary blood flow due to a left-to-right shunt. The importance of Cp has gained attention because recent reports have shown that low Cp potentially reflects poor prognosis in patients with CHD-PH and idiopathic pulmonary hypertension. It is also known that Cp levels decrease in specific populations, such as preterm infants and trisomy 21. Therefore, both Rp and Cp should be individually evaluated in the management of children with CHD-PH who have different disease conditions.

Role of CT and MRI in Cardiac Emergencies.

Current strategies for the evaluation of patients with chest pain have significantly changed thanks to the implemented potentiality of CT and MRI. The possible fatal consequences and high malpractice costs of missed acute coronary syndromes lead to unnecessary hospital admissions every year. CT provides consistent diagnostic support, mainly in suspected coronary disease in patients with a low or intermediate pre-test risk. Moreover, it can gain information in the case of cardiac involvement in pulmonary vascular obstructive disease. MRI, on the other hand, has a leading role in the condition of myocardial damage irrespective of the underlying inflammatory or stress related etiology. This article discusses how radiology techniques (CT and MRI) can impact the diagnostic workflow of the most common cardiac and vascular pathologies that are responsible for non-traumatic chest pain admissions to the Emergency Department.

Percutaneous Pulmonary Angioplasty for Patients With Takayasu Arteritis and Pulmonary Hypertension

BackgroundPercutaneous transluminal pulmonary angioplasty (PTPA) is a treatment modality for chronic thromboembolic pulmonary hypertension, but whether it can be applied to Takayasu arteritis–associated pulmonary hypertension (TA-PH), another chronic obstructive pulmonary vascular disease, remains unclear. ObjectivesThis study sought to investigate the efficacy and safety of PTPA for TA-PH. MethodsBetween January 1, 2016, and December 31, 2019, a total of 50 patients with TA-PH who completed the PTPA procedure (the PTPA group) and 21 patients who refused the PTPA procedure (the non-PTPA group) were prospectively enrolled in this cohort study. The primary outcome was all-cause mortality. The safety outcomes included PTPA procedure-related complications. ResultsBaseline characteristics and medical therapies were similar between the PTPA group and the non-PTPA group. During a mean follow-up time of 37 ± 14 months, deaths occurred in 3 patients (6.0%) in the PTPA group and 6 patients (28.6%) in the non-PTPA group, contributing to the 3-year survival rate of 93.7% in the PTPA group and 76.2% in the non-PTPA group (P = 0.0096 for log-rank test). The Cox regression model showed that PTPA was associated with a significantly reduced hazard of all-cause mortality in TA-PH patients (HR: 0.18; 95% CI: 0.05-0.73; P = 0.017). No periprocedural death occurred. Severe complications requiring noninvasive positive pressure ventilation occurred in only 1 of 150 total sessions (0.7%). ConclusionsPTPA tended to be associated with a reduced risk of all-cause mortality with acceptable safety profiles and seemed to be a promising therapeutic option for TA-PH patients.

Cardiovascular Diseases Associated with Pregnancy: An Overview

Valvular heart disease is the most common cardiac problem complicating pregnancy, and pregnancy in most women with heart disease has a favourable maternal and fetal outcome. With the exception of patients with Eisenmenger syndrome, pulmonary vascular obstructive disease, and Marfan syndrome with aortopathy, maternal death during pregnancy in women with heart disease is rare. However, pregnant women with heart disease do remain at risk for other complications including heart failure, arrhythmia, and stroke. Women with congenital heart disease now comprise the majority of pregnant women with heart disease seen at referral centres. The next largest group includes women with rheumatic heart disease. Peripartum cardiomyopathy, though infrequent, will be discussed in view of its unique relation to pregnancy. Two groups of conditions not discussed further are coronary artery disease which is infrequently encountered, and isolated mitral valve prolapse, which generally has an excellent outcome. Hormonally mediated increases in blood volume, red cell mass, and heart rate result in a major increase in cardiac output during pregnancy; cardiac output peaks during the second trimester, and remains constant until term. Gestational hormones, circulating prostaglandins, and the low resistance vascular bed in the placenta result in concomitant decreases in peripheral vascular resistance and blood pressure. During labour and delivery, pain and uterine contractions result in additional increases in cardiac output and blood pressure. Immediately following delivery, relief of caval compression and autotransfusion from the emptied and contracted uterus produce a further increase in cardiac output. Most haemodynamic changes of pregnancy resolve by two weeks postpartum.

Klotho as Potential Autophagy Regulator and Therapeutic Target.

The protein Klotho can significantly delay aging, so it has attracted widespread attention. Abnormal downregulation of Klotho has been detected in several aging-related diseases, such as Alzheimer’s disease, kidney injury, cancer, chronic obstructive pulmonary disease (COPD), vascular disease, muscular dystrophy and diabetes. Conversely, many exogenous and endogenous factors, several drugs, lifestyle changes and genetic manipulations were reported to exert therapeutic effects through increasing Klotho expression. In recent years, Klotho has been identified as a potential autophagy regulator. How Klotho may contribute to reversing the effects of aging and disease became clearer when it was linked to autophagy, the process in which eukaryotic cells clear away dysfunctional proteins and damaged organelles: the abovementioned diseases involve abnormal autophagy. Interestingly, growing evidence indicates that Klotho plays a dual role as inducer or inhibitor of autophagy in different physiological or pathological conditions through its influence on IGF-1/PI3K/Akt/mTOR signaling pathway, Beclin 1 expression and activity, as well as aldosterone level, which can help restore autophagy to beneficial levels. The present review examines the role of Klotho in regulating autophagy in Alzheimer’s disease, kidney injury, cancer, COPD, vascular disease, muscular dystrophy and diabetes. Targeting Klotho may provide a new perspective for preventing and treating aging-related diseases.

Percutaneous transluminal pulmonary angioplasty for hypertensive pulmonary Takayasu's arteritis

Abstract Background Percutaneous transluminal pulmonary angioplasty (PTPA) is a treatment modality for chronic thromboembolic pulmonary hypertension, but whether it can be applied to patients with hypertensive pulmonary Takayasu's arteritis (PTA), another chronic obstructive pulmonary vascular disease, remains unclear. Purpose To investigate the potential efficacy and safety of PTPA for hypertensive PTA. Methods All consecutive patients with hypertensive PTA who completed PTPA in our center between January 1, 2016 and December 31, 2019 were prospectively enrolled. Hemodynamics, exercise capacity and oxygen saturation were measured at baseline and at least 3 months after the last PTPA session. PTPA procedure related complications, restenosis and all-cause deaths during follow-up were recorded. Results A total of 50 patients were enrolled, with the median age of 41 (IQR, 30–49) years and female predominance (82.0%). After PTPA, significant improvements in hemodynamics including mean pulmonary artery pressure (from median 48 [39–60] to 37 [31–42] mm Hg, p<0.001) and pulmonary vascular resistance (from 9.6 [7.0–11.8] to 4.8 [3.6–6.4] Wood U, p<0.001) were achieved. Furthermore, PTPA also significantly improved the WHO functional class (p<0.001), six-minute walking distance (from 412 [313–470] to 492 [430–539] meters, p<0.001) and arterial oxygen saturation (from 91.3 [86.9–94.1] to 93.9 [91.8–95.3] %, p<0.001). No peri-procedural death occurred. The most common complication was pulmonary artery injury (27/150 PTPA sessions, 18.0%), which was always asymptomatic and without further clinical sequelae. Pulmonary angiography during follow-up revealed complete self-healing in 26 out of 27 (96.3%) vessels. Restenosis occurred in 5 out of 360 treated vessels (1.4%). Three patients died during follow-up, and the 1-year, 2-year and 3-year survival rates were 98.0%, 95.7% and 92.8%, respectively. Conclusion PTPA appears to be a promising therapeutic option for patients with hypertensive PTA. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): the National Key Research and Development Program of China Efficacy of PTPA

Predicting 10-year stroke mortality: development and validation of a nomogram

Predicting long-term stroke mortality is a clinically important and unmet need. We aimed to develop and internally validate a 10-year ischaemic stroke mortality prediction score. In this UK cohort study, 10,366 patients with first-ever ischaemic stroke between January 2003 and December 2016 were followed up for a median (interquartile range) of 5.47 (2.96–9.15) years. A Cox proportional-hazards model was used to predict 10-year post-admission mortality. The predictors associated with 10-year mortality included age, sex, Oxfordshire Community Stroke Project classification, estimated glomerular filtration rate (eGFR), pre-stroke modified Rankin Score, admission haemoglobin, sodium, white blood cell count and comorbidities (atrial fibrillation, coronary heart disease, heart failure, cancer, hypertension, chronic obstructive pulmonary disease, liver disease and peripheral vascular disease). The model was internally validated using bootstrap resampling to assess optimism in discrimination and calibration. A nomogram was created to facilitate application of the score at the point of care. Mean age (SD) was 78.5 ± 10.9 years, 52% female. Most strokes were partial anterior circulation syndromes (38%). 10-year mortality predictors were: total anterior circulation stroke (hazard ratio, 95% confidence intervals) (2.87, 2.62–3.14), eGFR < 15 (1.97, 1.55–2.52), 1-year increment in age (1.04, 1.04–1.05), liver disease (1.50, 1.20–1.87), peripheral vascular disease (1.39, 1.23–1.57), cancers (1.37, 1.27–1.47), heart failure (1.24, 1.15–1.34), 1-point increment in pre-stroke mRS (1.20, 1.17–1.22), atrial fibrillation (1.17, 1.10–1.24), coronary heart disease (1.09, 1.02–1.16), chronic obstructive pulmonary disease (1.13, 1.03–1.25) and hypertension (0.77, 0.72–0.82). Upon internal validation, the optimism-adjusted c-statistic was 0.76 and calibration slope was 0.98. Our 10-year mortality model uses routinely collected point-of-care information. It is the first 10-year mortality score in stroke. While the model was internally validated, further external validation is also warranted.

Tumor-Related and Patient-Related Variables Affecting Length of Hospital Stay Following Vestibular Schwannoma Microsurgery.

Review a single institution's vestibular schwannoma (VS) microsurgery experience to determine (1) correlations between demographics, comorbidities, and/or surgical approach on hospital length of stay (LOS) and discharge disposition and (2) trends in surgical approach over time. Retrospective case series from a multidisciplinary skull base program at a tertiary care, academic hospital. All adult (>18 years) patients undergoing primary microsurgery for VS between 2008 and 2018 were included. A total of 147 subjects were identified. Surgical approach was split between middle fossa (MF) (16%), retrosigmoid (RS) (35%), and translabyrinthine (TL) (49%) craniotomies. For the 8% of patients had other than routine (OTR) discharge. Mean LOS was significantly longer for patients undergoing RS than either MF or TL. Brainstem compression by the tumor was associated with longer LOS as were diagnoses of chronic obstructive pulmonary disease (COPD) and peripheral vascular disease (PVD). For all discharges, the 40 to 50- and 50 to 60-year-old subgroups had significantly shorter LOS than the 70-years-and-older patients. For the 92% of patients routinely discharged, there was a significantly shorter LOS in the 40 to 50-year-olds compared to the 70-years-and-older patients. There was a significant shift in surgical approach from RS to TL over the study period. Over 90% of VS microsurgery patients were routinely discharged with a median hospital LOS of 3.2 days, both of which are consistent with published data. There is an inverse relationship between age and LOS with patients older than 70 years having significantly longer LOS. Brainstem compression, COPD, PVD, and the RS approach negatively affect LOS. 4.

Real-world burden of myelosuppression in patients with small cell lung cancer (SCLC): Retrospective, longitudinal data analysis.

e19300 Background: Myelosuppression is a common side effect of chemotherapy (CT) that can compromise patient (pt) and economic outcomes. To evaluate the overall burden of myelosuppression, a longitudinal, patterns of care analysis was conducted among pts with SCLC treated with CT in routine clinical practice. Methods: Data were obtained from Providence St. Joseph Health electronic medical records between Jan 2016 and Dec 2019. Hematologic adverse events, treatment patterns, and hospital-based healthcare resource utilization and costs were assessed during the 12 months from first diagnosis of SCLC in pts who had CT-induced grade 3/4 myelosuppression in 1st or 1st/2nd/3rd-line (1/2/3L) treatment settings. Descriptive statistics were utilized to summarize key findings. Costs of care were calculated from actual treatment costs for inpatient, outpatient, and emergency room services after first diagnosis and treatment with CT. Professional billing was not included due to variability in physician employment by the system. Results: 347 pts were eligible for analysis; mean age (SD) was 66 (9.0) years, 49% were female, and 89% were Caucasian. Prominent comorbidities included chronic obstructive pulmonary disease (52%), diabetes (23%), and peripheral vascular disease (20%). 264 pts (76%) received 1L treatment only, while an additional 83 pts (24%) had both 1L and 2/3L treatment. Of 339 evaluable pts with longitudinal laboratory data, grade 3/4 cytopenias were reported as follows: 45%, neutropenia; 41%, anemia; and 25%, thrombocytopenia. 43% and 15% of pts received red blood cell or platelet transfusions, respectively. 49% of pts received prophylaxis (6%) or treatment (43%) with G-CSF, and 4% of pts were treated with erythropoiesis-stimulating agents. Average total costs of care (post initial-diagnosis and treatment) for pts without grade 3/4 hematologic events (n = 110) were $67,802 per pt throughout the 12 months’ follow-up. On average, annual per pt costs for those with grade 3/4 hematologic events were $131,047 for neutropenia, $95,954 for anemia, and $90,053 for thrombocytopenia. Conclusions: A large and meaningful proportion of pts had grade 3/4 myelosuppression with annual incremental associated costs per pt ranging from $22,251 for pts with thrombocytopenia to $63,245 for pts with neutropenia. Despite the availability of protocols of care and various rescue treatments, CT-induced myelosuppression places a significant real-world burden on pts and the healthcare system.

Eating Cheerios on ECMO: The Use of VA ECMO as a Bridge to Lung Transplant in an Infant

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. In PVOD the pulmonary venous vessels are obliterated resulting in obstructive pulmonary vascular disease, increase in pulmonary vascular resistance, right heart failure and death. Pulmonary hypertension is the most common reason for lung transplant in infants. Lung transplant is the only therapy for PVOD. Prolonged intubation, deconditioning and extra corporeal life support are associated with poor outcomes after lung transplant. We present a case of PVOD in an infant who had successful lung transplant using a novel veno-arterial extracorporeal membrane oxygenation (VA ECMO) configuration. A previously healthy 15-month-old male presented with cough and tachypnea. Cardiomegaly and pulmonary edema were seen on chest x-ray. Echocardiogram revealed severe pulmonary hypertension. Cardiac catheterization study and chest computed tomography revealed PVOD. He was unable to be extubated after cardiac catheterization and was transferred to our institution for lung transplant evaluation. He had severely depressed right ventricular function, requiring vasopressors to maintain hemodynamic stability. Respiratory status was tenuous, requiring sedation and paralysis. He was evaluated and listed for lung transplant. The patient was centrally cannulated for VA ECMO using cannulae for a ventricular assist device (Berlin ExCor) that were exteriorized on the abdomen. "Venous" inflow cannula was placed in the main pulmonary artery to provide afterload reduction for the right ventricle. After starting VA ECMO, vasopressor support, respiratory support, paralysis and sedation were weaned. He was woken up, extubated; and intensive physical and occupational rehabilitation began. He regained strength, was able to sit, stand and take steps. He also regained his milestones, was able to babble, wave and eat simple foods. The patient remained on VA ECMO for 61 days until he successfully underwent lung transplantation. Our case highlights the successful use of a novel VA ECMO configuration as a bridge to lung transplant in an infant with PVOD. To our knowledge this is the youngest patient to undergo awake VA ECMO reported. The use of a unique cannulation allowed for rehabilitation while on VA ECMO. Though challenging, VA ECMO remains an option as a bridge to lung transplant in infants with PVOD.

Pulmonary Vascular Obstructive Disease Prevention: A Case Report of Management Approach in CAVSD with large PDA

Background: Complete atrioventricular septal defect (CAVSD) and large patent ductus arteriosus (PDA) are considered as one of special conditionwhich need attention regarding its diagnosis and management. When CAVSD and large PDA is concomitant, the management is different from the simple case of CAVSD.&#x0D; Objective: Early recognition and optimal management of CAVSD and large PDA in area with limited medical facilities.&#x0D; Methods: This study will be presented as a case report.&#x0D; Case Illustration: A twenty-days-old female neonate was born with CAVSD and large PDA. The baby was in the 37 weeks gestational age when she was born spontaneously with APGAR score&#x0D; 8/9 and birth weight of 2890 g. Conservative approach was preferred as the patient was treated with captopril 0.8 mg three times a day, spironolactone2mg twice a day and hydrochlorothiazide 1 mg twice a day to prevent heart failure.&#x0D; Conclusion: Pre-ductal and post-ductal oximetry test measurement is a reliable approach to screen patient with congenital heart disease which might be applied in medical center with limited medical facilities. Moreover, regarding the management, it might be beneficial to conduct early pulmonary artery banding.

Суживание легочной артерии при врожденных пороках сердца с легочной гипертензией и легочно-сосудистой болезнью

Суживание легочной артерии при врожденных пороках сердца с легочной гипертензией и легочно-сосудистой болезнью

Паллиативное предсердное и артериальное переключение при транспозиции магистральных артерий с дефектом межжелудочковой перегородки и тяжелой легочно-сосудистой болезнью

Паллиативное предсердное и артериальное переключение при транспозиции магистральных артерий с дефектом межжелудочковой перегородки и тяжелой легочно-сосудистой болезнью

Chronic Obstructive Pulmonary Disease and Pulmonary Vascular Disease. A Comorbidity?

Chronic obstructive pulmonary disease (COPD) is one of the most important causes of death worldwide, and in addition to its impact on the patient's health, it poses a major socioeconomic burden. Tobacco smoke, indoor cooking, and air pollution are major triggers of the disease. This article summarizes evidence for the concept that lung microvascular molecular alterations can be a driver of lung emphysema. If findings from preclinical models allow a transfer to the human situation, this concept can offer new approaches for curative treatment of lung emphysema.

Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects.

This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.

Modelling Bronchopulmonary Dysplasia in Animals: Arguments for the Preterm Rabbit Model.

Bronchopulmonary dysplasia (BPD) remains a frequent and disabling consequence of preterm birth, despite the recent advances in neonatal intensive care. There is a need to further improve outcomes and many novel therapeutic or preventive strategies are therefore investigated in animal models. We discuss in this review the aspects of human BPD pathophysiology and phenotype, which ideally should be mimicked by an animal model for this disease. Prematurity remains the common denominator in the heterogeneous spectrum of human BPD, and preterm animal models thus have a clear translational advantage. Additional factors, like excessive oxygen, mechanical ventilation and infection, which frequently have been studied in animal models, can contribute to preterm lung injury however are not indispensable to develop BPD. The phenotype of human BPD is characterized by alveolar developmental arrest with extracellular matrix remodeling, signs of obstructive airway disease and pulmonary vascular disease. Many animal models mimic this phenotype and have their place in BPD research, but results should be interpreted bearing in mind the specific advantages and disadvantages of the model. Term mice and rats are well suited for basic explorative research on specific disease mechanisms, essential for the generation of new hypotheses, while the larger ventilated preterm baboons and lambs provide a good platform for the ultimate translation of these strategies towards clinical application. The preterm rabbit model seems a promising model as it the smallest model that includes a factor of prematurity and has a unique position between the small and large animal models.

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

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.

External stenting: A reliable technique to relieve airway obstruction in small children

ObjectiveAirway obstruction in children may be caused by conditions such as vascular compression and congenital tracheobronchomalacia. Obstructive pulmonary vascular disease may be a detrimental sequel for patients with congenital heart disease. We evaluate our own original external stenting technique as a treatment option for these patients. MethodsNinety-eight patients underwent external stenting (1997-2015). Cardiovascular anomalies were noted in 82 (83.7%). Nine patients had hypoplastic left heart syndrome and 6 had other types of single-ventricular hearts. ResultsThe median age at the first operation was 7.2 months (range, 1.0-77.1 months). The mechanisms were tracheobronchomalacia with (n = 46) or without (n = 52) vascular compression. Patients underwent 127 external stentings for 139 obstruction sites (62 trachea, 55 left bronchus, and 22 right bronchus). The stent sizes varied from 12 to 16 mm. There were 14 (8 in the hospital and 6 after discharge) mortality cases. Nine required reoperation for restenosis and 3 required stent removal for infection. The actuarial freedom from mortality and any kind of reoperation was 74.7% ± 4.6% after 2.8 years. The negative pressure threshold to induce airway collapse for congenital malacia (n = 58) improved from −15.9 to −116.0 cmH2O. A follow-up computed tomography scan (>2.0 years interval from the operation; n = 23) showed the mean diameter of the stented segment at 88.5% ± 13.7% (bronchus) and 94.5% ± 8.2% (trachea) of the reference. ConclusionsExternal stenting is a reliable method to relieve airway compression for small children, allowing an age-proportional growth of the airway.