Severe pulmonary hypertension as a diagnostic challenge: a case of congenitally corrected transposition of the great arteries with dextrocardia in an elderly patient.

Survival Benefit of Aortic Valve Replacement in Patients With Severe Aortic Regurgitation and Pulmonary Hypertension

RationaleIdiopathic Pulmonary Fibrosis (IPF) is a rapidly progressive fatal disease when compounded with Pulmonary Hypertension has a mortality rate over 50% within 5 years of diagnosis. The only effective therapy for advanced interstitial lung disease shown to improve quality of life and survival is lung transplantation. Although bilateral lung transplantation is often performed in IPF with severe pulmonary hypertension, it is essential to evaluate the short and long term outcomes of patients with single lung transplants and assess if this is a viable option. In this study we investigated the short team outcomes in patients receiving both single and double lung transplantation stratified by their Pulmonary Vascular Resistance (PVR) using the 2022 ESC/ERS diagnostic criteria for severe pulmonary hypertension defined as a PVR Greater than 5 MethodsAfter receiving IRB approval at Temple University (IRB # 29339-0001) we performed a retrospective study of lung transplant recipients using the International Society of Heart and Lung Transplant (ISHLT)/UNOS research registry between January 2008 and June 2018. Isolated adult lung transplant recipients for IPF were identified. All patients receiving a re-transplantation were excluded. Demographic data were compiled and described. Baseline characteristics and outcomes were compared. Patient's were stratified into Severe (PVR>5) versus non-severe (PVR<5) pulmonary hypertension. ResultsA total of 7048 patients were identified. Of them, 6453 (91.5%) of patients were found to have non severe pulmonary hypertension (PVR<5) and 595 (8.5 %) patients were found to have severe pulmonary hypertension (PVR >5). For the patients with severe pulmonary hypertension, statistically significant differences noted were a younger patient population, higher percentage of patients who received double lung transplantation, a higher FVC percentage predicted, higher pre-transplant need for mechanical ventilation, increased cardiopulmonary hemodynamics at time of listing for transplant, and an increased ischemic time. Using Cox regression, in patients receiving a single lung transplant, 1 year survival was 85.9% for non-severe pulmonary hypertension and 81.5% survival for Severe pulmonary hypertension. In double lung transplant, 1 year survival was 87.3% for patients with non-severe pulmonary hypertension and 86.1% in patients with severe pulmonary hypertension. ConclusionWhile bilateral lung transplant remains superior to single lung transplant in terms of long-term survival, single lung transplantation may be an acceptable therapeutic option for patients with Idiopathic Pulmonary Fibrosis with Pulmonary Hypertension. As the number of patients with IPF awaiting lung transplantation continues to grow, single lung transplant may help overall improve survival in patients with a rapidly progressive deadly disease.

Aim:Long standing mitral valve disease is usually associated with severe pulmonary hypertension. Perioperative pulmonary hypertension is a risk factor for right ventricular (RV) failure and a cause for morbidity and mortality in patients undergoing mitral valve replacement. Phosphodiesterase 5 inhibitor-sildenafil citrate is widely used to treat primary pulmonary hypertension. There is a lack of evidence of effects of oral sildenafil on secondary pulmonary hypertension due to mitral valve disease. The study aims to assess the effectiveness of preoperative oral sildenafil on severe pulmonary hypertension and incidence of RV failure in patients undergoing mitral valve replacement surgery.Materials and Methods:A total of 40 patients scheduled for mitral valve replacement with severe pulmonary hypertension (RV systolic pressure (RVSP) ≥60 mmHg) on preoperative transthoracic echo were randomly treated with oral sildenafil 25 mg (N = 20) or placebo (N = 20) eight hourly for 24 h before surgery. Hemodynamic variables were measured 20 min after insertion of pulmonary artery catheter (PAC) under anesthesia (T1), 20 min at weaning from cardiopulmonary bypass (CPB) (T2) and after 1,2, and 6 h (T3, T4, T5, respectively) during the postoperative period.Results:Systolic and mean pulmonary artery pressure (MPAP) and pulmonary vascular resistance index (PVRI) were significantly lower (P < 0.0001) in sildenafil group at all times. Ventilation time and postoperative recovery room stay were significantly lower (P < 0.001) in sildenafil group.Conclusion:Sildenafil produces significant pulmonary vasodilatory effect as compared with placebo in mitral valve replacement patients with severe pulmonary hypertension. It also reduces ventilation time and intensive care unit (ICU) stay time as compared with placebo. It is concluded that sildenafil is effective in reducing pulmonary hypertension when administered preoperatively in patients with severe pulmonary hypertension undergoing mitral valve replacement surgery.

ABSTRACTPartial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous circulation. PAPVC is often misdiagnosed as idiopathic pulmonary hypertension in the adult population. We report a case of a 48‐year‐old woman with long‐standing pulmonary hypertension who presented with recurrent pulmonary oedema and right‐sided heart failure. Transthoracic echocardiography showed severe pulmonary hypertension with preserved left ventricular function. Computed tomographic pulmonary angiography excluded pulmonary embolism but confirmed an anomalous drainage of the right upper pulmonary vein into the superior vena cava, consistent with isolated PAPVC without an atrial septal defect. Right heart catheterization confirmed severe precapillary pulmonary hypertension with a positive vasoreactivity response to adenosine. She was successfully managed medically with diltiazem and diuretics, showing sustained clinical improvement. This case highlights isolated PAPVC as a critical, under‐recognised cause of severe pulmonary hypertension in adults.

Aortic Valve Replacement Improves Survival in Severe Aortic Stenosis Associated With Severe Pulmonary Hypertension

The Cardio-Obstetrics Patient and the Cardiothoracic Anesthesiologist

Going Home with a Patent Ductus Arteriosus: Is it Benign?

Pulmonary hypertension is defined as resting arterial pressure >20 mmHg. Cardiac tamponade is a medical emergency where fluids accumulate in the pericardial sac compressing the heart pericardium leading to heart failure. Pericardiocentesis is challenging in patients with cardiac tamponade and severe pulmonary hypertension due to the risk of catastrophic haemodynamic collapse. An 18-year-old female who was recently diagnosed to have systemic lupus erythematosus (SLE) presented to the emergency department with shortness of breath, chest pain, fever, and fatigue. The physical examination revealed tachycardia, muffled heart sounds, and distended jugular venous pulse. Chest X-ray showed cardiomegaly, and transthoracic echocardiography showed a large circumferential pericardial effusion with signs of cardiac tamponade. There was severe pulmonary hypertension along with a dilated right ventricle with systolic dysfunction. The right ventricular systolic pressure was around 100 mmHg. The multidisciplinary team of cardiologists and pulmonologists decided to avoid pericardiocentesis due to the high risk of haemodynamic collapse. Aggressive medical therapy targeting pulmonary hypertension and SLE was started, which resulted in complete resolution of the pericardial effusion and normalization of pulmonary artery pressure. A conservative approach can be an alternative strategy to manage patients with large pericardial effusion and impending pericardial tamponade in the presence of severe pulmonary arterial hypertension as pericardiocentesis carries a high risk of haemodynamic collapse.

Severe pulmonary hypertension occurs occasionally in patients with chronic obstructive pulmonary disease (COPD), but no detailed description of these patients is available. To identify and characterize patients with COPD and severe pulmonary hypertension. Retrospective study of 27 patients with COPD with severe pulmonary hypertension (pulmonary artery mean pressure [Ppa], > or = 40 mm Hg) among 998 patients who underwent right heart catheterization between 1990 and 2002 as part of a workup for chronic respiratory failure during a period of disease stability. Of the 27 patients, 16 had another disease capable of causing pulmonary hypertension. The remaining 11 (11 of 998, 1.1%) patients had COPD as the only cause of pulmonary hypertension, with a median Ppa of 48 mm Hg (interquartile range, 46-50). They had an unusual pattern of cardiopulmonary abnormalities with mild to moderate airway obstruction, severe hypoxemia, hypocapnia, and a very low diffusing capacity for carbon monoxide (p < 0.01 compared with a control group of patients with COPD). Exertional dyspnea was more severe (p < 0.01) and survival was shorter (p = 0.0026) than in the control subjects. Severe pulmonary hypertension is uncommon in patients with COPD. When it occurs, another cause must be sought. COPD with severe pulmonary hypertension and no other possible cause shares features with pulmonary vascular diseases, such as idiopathic pulmonary hypertension.

Purpose To study the clinical echocardiographic features of various variants of pulmonary hypertension (PH). Methods of research. Clinical evaluation and analysis of the results of 45,000 Doppler echocardiographic studies were performed. Moderate PH corresponded to the mean pulmonary artery pressure (MPAP) level up to 50 mm Hg, severe PH was characterized by MPAP above 50 mm Hg. Results Clinically significant moderate and severe PH was established in 283 patients, which was 0.63% of the examined patients. Severe PH was detected in 106 patients, including 30 men and 76 women, whose average age was 43.1 ± 1.7 years in the age range of 14-75 years. Patients with congenital heart disease (CHD) represented the majority of the patients with severe pulmonary arterial hypertension (PAH) - 49 people (10 men and 39 women, mean age 43.0 ± 1.8 years in the age range of 14-69 years), including 16 with ventricular septal defect, 9 with an atrial septal defect, 7 with transposition of the great arteries, 6 with complete common atrioventricular canal defect, 3 with congenitally corrected transposition of the great arteries, 3 with a double outlet right ventricle, 3 with a common arterial trunk, 2 with the Ebstein anomaly. Severe PH was also established in 12 patients with idiopathic PAH, 21 patients with recurrent pulmonary embolism (PE), 10 with acquired valvular heart disease (AVHD) (in 7 cases with severe mitral stenosis, 3 with severe aortic stenosis), in 7 patients with postinfarction left ventricular aneurysm, 4 patients with chronic obstructive pulmonary disease (COPD), 3 with dilated cardiomyopathy. Moderate PH was diagnosed in 177 patients (97 men and 80 women, mean age 60.3 ± 1.3 years in the age range of 21-87 years), including 54 patients with dilated, 26 - ischemic cardiomyopathy, 38 with AVHD, 26 with PE, 17 post-MI patients, 9 with CHD, and 7 with COPD. PAH-specific therapy with bosentan and / or sildenafil was performed in 9 patients with idiopathic PAH and 7 patients with CHD without significant clinical effect; in other cases, nonspecific therapy was continued with diuretics, positive inotropic agents, and anticoagulants. Conclusions The most pronounced manifestations of PH were observed predominantly in CHD patients with advanced left-to-right and right-to-left shunting of blood, idiopathic PAH and thromboembolic PH. For prevailing venous PH, the less pronounced clinical manifestations were observed in patients with ischemic, non-coronary and valvular heart lesions, as well as with COPD accompanied by hypoxemia.

Objective Investigate the clinical features, diagnosis and treatments of the scimitar syndrome, and different forms of treatment to alleviate pulmonary hypertension. Methods A retrospective analysis of clinical data of 14 children with scimitar syndrome from 2013 to 2017, including clinical symptoms and signs, chest X ray, echocardiography, chest CT and cardiac catheterization, treatment outcome and follow-up. Assess embolization of systemic pulmonary collateral and pulmonary venous drainage correction surgery, which is better for lowering pulmonary blood flow. Results 14 patients with scimitar syndrome were diagnosed from 2013 to 2017. There were 5 boys and 9 girls; 3 cases<7 kg in weight. Scimitar syndrome was suspected because of extroversion, and diagnosed by color Doppler echocardiography and 13 of them confirmed by cadiac CT scan when ascimitar vein was detected entering the inferior vena cava. 11 patiens had right lung dysplasia and 4 had horseshoe lung. Three patients had severe pulmonary arterial hypertension, 3 had moderate to severe pulmonary arterial hypertension, and 2 had moderate pulmonary arterial hypertension, the left had slight pulmonary arterial hypertension. 4 patients had pulmonary venous drainage correction surgery, after that 2 of them had systemic pulmonary collateral embolism. 6 patients systemic pulmonary collateral embolism first, then 4 of them had surgical repair, 1 case of 13 years old asymptomatic child without surgery. 1 patient with heart failure, severe pulmonary hypertension, pulmonary infection, died before surgery, while another died after surgical repair. At last 1 patient was lost for follow-up visits. Systemic pulmonary collateral embolism and pulmonary venous drainage correction surgery could all reduce blood flow of pulmonary. After systemic pulmonary collateral embolism, patients had slight pulmonary arterial hypertension just need follow-up visits. Conclusion Clinically, found children with heart of dextrocardia position, growth retardation, recurrent lung infections, unexplained right heart failure, pulmonary hypertension, should consider the possibility scimitar syndrome. Whether pulmonary vascular embolization or surgical repair, can significantly reduce pulmonary artery’s blood flow and alleviate pulmonary hypertension to protect pulmonary, even reduced the incidence of pneumonia and mortality. So we suppose ealy pulmonary hypertension in scimitar syndrome patients maybe dynamic pulmonary hypertension. Key words: Congenital heart defect; Scimitar syndrome; Pulmonary vein; Pulmonary hypertension; Systemic pulmonary collateral circulation

Considering the important surface in pulmonary circulation where blood can interact with the endothelium, the maintenance of blood fluidity through the lung, by antithrombotic pathways and products of the endothelium, is essential. This function appears to be ineffective in primary pulmonary hypertension and in severe secondary pulmonary hypertension. Thrombotic lesions are frequently found in pulmonary arteries in these diseases. Thrombin activity appears to be increased in severe pulmonary hypertension. Antithrombotic pathway disorders may account for this abnormality, particularly in chronic thromboembolic pulmonary hypertension and primary pulmonary hypertension. Injured endothelium, a constant feature in severe pulmonary hypertension, either primary or secondary, enhances thrombus formation in pulmonary vessels. This is probably related to thrombomodulin and tissue factor imbalance, impairment of prostacyclin and nitric oxide release, as well as inefficiency of fibrinolysis. Moreover, platelets appear to be activated in the pulmonary circulation of these patients. They release several mediators acting on vascular tone and as mitogenic agents, and may also contribute to thrombin and clot generation. Long-term oral anticoagulant and continuous infusion of prostacyclin, treatments which impede thrombosis, are known to improve the survival rate in patients with primary pulmonary hypertension. These are the strongest arguments, so far, in favour of the role of thrombosis in severe pulmonary hypertension. However, we do not know whether these abnormalities result from a previous vascular injury or represent the primary disturbance.

In patients with severe pulmonary arterial hypertension, subcutaneous or catheter-based intravenous application of prostanoids carries a risk of local side effects or systemic infections, which limits their use and acceptance. Recently, a fully implantable pump for continuous application of intravenous treprostinil was approved in Germany. However, surgery is a major risk for patients with severe pulmonary arterial hypertension. The purpose of this study was to investigate the safety of a fully implantable pump inserted under local or general anesthesia in patients with severe pulmonary hypertension. All patients with pulmonary hypertension undergoing pump implantation for the continuous application of intravenous treprostinil were included from two German centers. Surgery was performed under local or general anesthesia according to the protocol of the recruiting center. Intra-operative safety and in-hospital complications were analyzed for the two different implantation regimens. In total, 51 patients were included. No major intra-operative complications were recorded. During the observation period, two patients died of progressive right heart failure, and two patients required treatment in the intensive care unit for acute right heart decompensation and respiratory failure. In total, major complications occurred in 8 out of 51 patients. Our observational study provides preliminary evidence supporting the procedural safety of a fully implantable pump inserted under local or general anesthesia for patients with severe pulmonary hypertension. The observation of major complications in a subset of patients requires extensive pre- and post-operative assessments. Future trials are required to provide further evidence for the long-term safety and efficacy of the pump using this approach.

Efficacy of Oral Sildenafil as Rescue Therapy in Patients With Severe Pulmonary Arterial Hypertension Chronically Treated With Prostacyclin. Long-Term Results

Pulmonary arterial hypertension is associated with a decreased antioxidant capacity. However, neither the contribution of reactive oxygen species to pulmonary vasoconstrictor sensitivity, nor the therapeutic efficacy of antioxidant strategies in this setting are known. We hypothesized that reactive oxygen species play a central role in mediating both vasoconstrictor and arterial remodeling components of severe pulmonary arterial hypertension. We examined the effect of the chemical antioxidant, TEMPOL, on right ventricular systolic pressure, vascular remodeling, and enhanced vasoconstrictor reactivity in both chronic hypoxia and hypoxia/SU5416 rat models of pulmonary hypertension. SU5416 is a vascular endothelial growth factor receptor antagonist and the combination of chronic hypoxia/SU5416 produces a model of severe pulmonary arterial hypertension with vascular plexiform lesions/fibrosis that is not present with chronic hypoxia alone. The major findings from this study are: 1) compared to hypoxia alone, hypoxia/SU5416 exposure caused more severe pulmonary hypertension, right ventricular hypertrophy, adventitial lesion formation, and greater vasoconstrictor sensitivity through a superoxide and Rho kinase-dependent Ca2+ sensitization mechanism. 2) Chronic hypoxia increased medial muscularization and superoxide levels, however there was no effect of SU5416 to augment these responses. 3) Treatment with TEMPOL decreased right ventricular systolic pressure in both hypoxia and hypoxia/SU5416 groups. 4) This effect of TEMPOL was associated with normalization of vasoconstrictor responses, but not arterial remodeling. Rather, medial hypertrophy and adventitial fibrotic lesion formation were more pronounced following chronic TEMPOL treatment in hypoxia/SU5416 rats. Our findings support a major role for reactive oxygen species in mediating enhanced vasoconstrictor reactivity and pulmonary hypertension in both chronic hypoxia and hypoxia/SU5416 rat models, despite a paradoxical effect of antioxidant therapy to exacerbate arterial remodeling in animals with severe pulmonary arterial hypertension in the hypoxia/SU5416 model.