Higher Incidence Of Pulmonary Hypertension Research Articles

Outcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database.

Controversial data exist about the impact of Down syndrome on outcomes after surgical repair of atrioventricular septal defect. (A) assess trends and outcomes of atrioventricular septal defect with and without Down syndrome and (B) determine risk factors associated with adverse outcomes after atrioventricular septal defect repair. We queried The National Inpatient Sample using International Classification of Disease codes for patients with atrioventricular septal defect < 1 year of age from 2000 to 2018. Patients' characteristics, co-morbidities, mortality, and healthcare utilisation were evaluated by comparing those with versus without Down syndrome. In total, 2,318,706 patients with CHD were examined; of them, 61,101 (2.6%) had atrioventricular septal defect. The incidence of hospitalisation in infants with atrioventricular septal defect ranged from 4.5 to 7.5% of all infants hospitalised with CHD per year. A total of 33,453 (54.7%) patients were associated with Down syndrome. Double outlet right ventricle, coarctation of the aorta, and tetralogy of Fallot were the most commonly associated with CHD in 6.9, 5.7, and 4.3% of patients, respectively. Overall atrioventricular septal defect mortality was 6.3%. Multivariate analysis revealed that prematurity, low birth weight, pulmonary hypertension, and heart block were associated with mortality. Down syndrome was associated with a higher incidence of pulmonary hypertension (4.3 versus 2.8%, p < 0.001), less arrhythmia (6.6 versus 11.2%, p < 0.001), shorter duration for mechanical ventilation, shorter hospital stay, and less perioperative mortality (2.4 versus 11.1%, p < 0.001). Trends in atrioventricular septal defect hospitalisation had been stable over time. Perioperative mortality in atrioventricular septal defect was associated with prematurity, low birth weight, pulmonary hypertension, heart block, acute kidney injury, and septicaemia. Down syndrome was present in more than half of atrioventricular septal defect patients and was associated with a higher incidence of pulmonary hypertension but less arrhythmia, lower mortality, shorter hospital stay, and less resource utilisation.

Pulmonary Artery Pressure Status in Hemodialysis Patients and Its Association with Nutritional and Biochemical Markers

Background: The prevalence of pulmonary hypertension (PH) is reported between 17% and 56% in hemodialysis patients. The pathogenesis of PH in hemodialysis patients is still unclear. Malnutrition may be associated with impaired pulmonary function in hemodialysis patients. Objectives: The present study aimed to investigate the association between PH and nutritional and biochemical markers in hemodialysis patients. Methods: In this cross-sectional study, 80 patients undergoing hemodialysis at the Kowsar Medical Center, Semnan, Iran, from 1 March 2016 to 31 August 2016 were enrolled by complete enumeration. Pulmonary artery pressure was determined by echocardiography between hemodialysis sessions. Arm circumference, triceps fat mass, and body mass index (BMI) were determined. Other laboratory tests included transferrin, serum albumin, cholesterol, triglyceride, and hemoglobin. The source of receiving protein was recorded in a questionnaire. The data were analyzed by the chi-square test and logistic regression analysis at a significance level of less than 0.05. Results: Pulmonary hypertension ( &gt; 25 mmHg) was found in 32 (40%) of the 80 patients studied. The mean of pulmonary pressure in patients with PH was 34.66 ± 5.6 mmHg compared to 16.85 ± 3.7 mmHg in patients without PH. Age, gender, arm circumference, frequency of dialysis per year, and serum levels of albumin, transferrin, triglyceride, and total cholesterol were not significantly different between patients with and without PH. There was a significant difference in the mean hemoglobin level between patients with and without PH (P = 0.003). The likelihood of developing PH was reduced by 0.3 in those with hemoglobin ≥ 11 g/dL compared to patients with hemoglobin levels below 11 g/dL (OR = 0.400, P = 0.003). The likelihood of developing PH in patients with albumin ≥ 3.8 g/L decreased by 0.126 compared to those with albumin less than 3.8 (OR = 0.126, P = 0.003). Conclusions: The results of our study indicated a higher incidence of PH in patients with lower levels of hemoglobin and albumin. So, patients undergoing hemodialysis are recommended to be regularly screened for these two variables.

Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Scimitar syndrome (SS) is a rare congenital condition which includes partial anomalous pulmonary venous return (PAPVR) and a variable degree of pulmonary hypoplasia. We describe the clinical features, therapeutic approach and outcomes of patients who underwent cardiac catheterization and/or surgical repair of the scimitar vein at a single institutionin the United States. This retrospective cohort study included all patients with SS who underwent scimitar vein surgical repair or cardiac catheterization from October 1989 through August 2021 in a tertiary care center. A total of 84 patients with SS were included and median follow-up time was 74months. Patients diagnosed with SS under the age of one year had a significantly greater incidence of congenital heart defects (CHD) (p < 0.001), non-cardiac anomalies (p = 0.02), pulmonary hypertension (p = 0.02), and mortality (p = 0.04) compared to those diagnosed over the age of 1year. Twenty-eight patients underwent surgical repair of the scimitar vein. Overall, eight (10%) patients died. Compared to surviving patients, deceased patients had a significantly higher incidence of pulmonary hypertension (PH), neonatal SS diagnosis, and extracorporeal membrane oxygenation (ECMO) support. Median scimitar vein pressure (20mmHg) of deceased patients was significantly higher compared to pressures in surviving patients (11mmHg; p = 0.02). PH, CHD, neonatal SS diagnosis, ECMO support, and markedly elevated scimitar vein pressure are associated with mortality. Scimitar vein surgical repair during infancy is commonly associated with PH and restenosis that requires re-intervention.

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Pediatric Restrictive Cardiomyopathies.

Restrictive cardiomyopathy (RCM) is the least frequent phenotype among pediatric heart muscle diseases, representing only 2.5–3% of all cardiomyopathies diagnosed during childhood. Pediatric RCM has a poor prognosis, high incidence of pulmonary hypertension (PH), thromboembolic events, and sudden death, is less amenable to medical or surgical treatment with high mortality rates. In this scenario, heart transplantation remains the only successful therapeutic option. Despite a shared hemodynamic profile, characterized by severe diastolic dysfunction and restrictive ventricular filling, with normal ventricle ejection fraction and wall thickness, RCM recognizes a broad etiological spectrum, consisting of genetic/familial and acquired causes, each of which has a distinct pathophysiology and natural course. Hence, the aim of this review is to cover the causes, clinical presentation, diagnostic evaluation, treatment, and prognosis of pediatric RCM.

Study of pulmonary arterial hypertension in patients of chronic kidney disease stage IV and V

Background: Pulmonary arterial hypertension (PAH) in advanced CKD patients (stage 4/5) is important to recognize, as PAH is an independent predictor of mortality in CKD patients especially those receiving renal replacement therapy and significant PH is felt to be a relative contraindication to renal transplantation. Present study was aimed to study Pulmonary Arterial Hypertension in Patients of Chronic Kidney Disease Stage 4/5 at a tertiary hospital. Material and Methods: Present study was cross-sectional, prospective and observational study, conducted patients Age ≥18 years, of either gender, diagnosed case of CKD in stage IV and stage V, underwent evaluation for PAH by echocardiography. Results: In present study, total 68 patients were included. Mean age in study was 51.93 ± 12.63 years. Male patients (73.53 %) outnumbered female patients (26.47 %). Associated Co-morbidities in present study were anemia (70.59 %), diabetes mellitus (57.35 %), systolic hypertension (32.35 %) and diastolic hypertension (26.47 %). majority of patients had stage 4 CKD (69.12 %). Mean duration of CKD was 43.11 + 26.78 weeks while mean duration of dialysis was 21.45± 15.74 weeks. 61.76 % patients had attained Dry weight in present study. In present study, incidence of pulmonary hypertension was 51.35 %. In patients with pulmonary hypertension most common etiology for CKD were diabetes mellitus (34.29%), hypertension (31.43 %), undetermined (17.14 %). In present study 47 patients (69.12 %) had stage 4 CKD. Among stage 4 patients mild PH was noted in 11 patients (16.18 %), while moderate and severe PH was noted in 4 (5.88 %) and 2 (2.94 %) patients respectively. While 21 patients (30.88 %) had stage 5 CKD. Among stage 5 patients mild PH was noted in 7 patients (10.19 %), while moderate and severe PH was noted in 5 (7.35 %) and 4 (5.88 %) patients respectively. Conclusion: Higher incidence of pulmonary hypertension is noted among chronic kidney disease and incidence increases with advanced stages.

Risk factors for the flare of systemic lupus erythematosus and its influence on prognosis: a single-center retrospective analysis

ObjectivesTo explore the risk factors for systemic lupus erythematosus (SLE) flare and their impact on prognosis.MethodsThe clinical characteristics, laboratory results, and treatment plans of 121 patients with SLE flare were retrospectively analyzed. Ninety-eight SLE outpatients with sustained remission during the same period were selected as controls. Logistic multivariate regression analysis was employed to screen for risk factors for SLE flare.ResultsInfection, thrombocytopenia, arthritis, anti-nucleosome antibodies positive, anti-β2-glycoprotein I (IgG) antibodies positive, and patient’s self-discontinuation of medicine maintenance therapy might be risk factors for SLE flare. Patients who discontinued medicine maintenance therapy by themselves had a significantly higher rate of severe SLE flare than patients with regular medicine maintenance therapy (P = 0.033). The incidence of anemia associated with SLE (P = 0.001), serositis (P = 0.005), and pulmonary hypertension (P = 0.003) in patients who discontinued medicine maintenance therapy were significantly higher than patients with regular medicine maintenance therapy. SLE patients with regular medicine maintenance therapy for less than 3 years had a higher risk of pulmonary hypertension than those with regular medicine maintenance therapy longer than 3 years (P = 0.034).ConclusionsThe accompanying thrombocytopenia, arthritis, anti-nucleosome antibodies positive and anti-β2-glycoprotein I (IgG) antibodies positive at the onset of SLE may affect the prognosis of SLE. Patient’s self-discontinuation of medicine maintenance therapy is the main cause of SLE flare, which may induce severe flare in SLE patients and lead to a significantly higher incidence of pulmonary hypertension.

Abstract 14185: Targeting Lower Oxygen Saturation Parameters in Premature Infants Does Not Increase Incidence of Pulmonary Hypertension in the Neonatal Intensive Care Unit

Introduction: Lower oxygen saturation targets in preterm infants have been associated with decreased incidence of bronchopulmonary dysplasia (BPD) but increased risk of pulmonary hypertension (PH). Studies have shown that targets of &lt;90% are associated with higher incidence of PH, however data on the optimal saturation target &gt;90% are lacking. In this study, we compared the rate of BPD and PH in two cohorts with saturation targets of 94-98% and 92-97%. We hypothesized that BPD rate would be lower and PH rate would be unchanged at the lower saturation target. Methods: We performed a retrospective cohort study comparing PH and BPD rates among two cohorts of infants born at ≤32 weeks gestation at Brigham and Women’s Hospital: cohort 1 with saturation target of 94-98% (n = 129, July 2017-July 2018), cohort 2 with saturation target of 92-97% (n = 124, July 2018-July 2019). PH was defined by echocardiographic evidence of systolic septal flattening or right ventricular pressure ≥35 mmHg (estimated by tricuspid regurgitant jet velocity or shunt velocity) at gestational age (GA) ≥36 weeks. Comparisons between groups were carried out by Chi-square test, t-test, and multivariable logistic regression. Results: Subjects had a GA of 23-32 weeks; 46% were female. Groups did not differ with respect to GA, sex, or birth weight. There was no difference in rate of PH (2.4% vs. 4.2%, p = 0.12) or BPD (25% vs. 20%, p = 0.31) between cohort 1 and cohort 2. Other clinical parameters were not different between groups, including presence of patent ductus arteriosus, presence of atrial septal defect, use of diuretics, or use of steroids. After controlling for GA, birth weight, sex, and diagnosis of BPD using logistic regression, there was no difference in rate of PH between groups (p = 0.47), but there was a positive association of BPD with PH (OR 3.45; 95% CI, 1.18-10.09; p = 0.02). Conclusions: A lower oxygen saturation target was not associated with a higher rate of PH or lower rate of BPD in preterm infants. The overall rate of PH was much lower than rates previously reported at saturation targets &lt;90%. Given our low incidence of PH, and the lack of a significant difference in rate of PH between groups, a saturation target of 92-97% may be safe while also minimizing need for supplemental oxygen in this population.

36. Scoliosis surgery normalizes cardiac function in AIS patients

BACKGROUND CONTEXT Spinal deformity in adolescent idiopathic scoliosis (AIS) can increase right atrial and ventricular pressures secondary to restrictive lung disease. Pulmonary hypertension leading to cor pulmonale is the most feared outcome; however, mild pulmonary hypertension in AIS patients has been reported in the past. No study has examined these changes including the improvement of right heart function following scoliosis surgery. PURPOSE To determine if AIS patients, compared to healthy patients, will have a higher incidence of pulmonary hypertension that worsens with increasing curve angle and improves following scoliosis surgery. STUDY DESIGN/SETTING Retrospective review. PATIENT SAMPLE A total of 202 healthy AIS patients undergoing posterior spinal fusion from 2009-2013 at a single institution (Group 1), 179 healthy age-matched control patients, who did not undergo spinal deformity correction, at the same institution (Group 2) and 47 AIS patients from with elevated preoperative tricuspid regurgitant jet velocity (TRV) undergoing posterior spinal fusion (PSF). OUTCOME MEASURES Right ventricular systolic pressure (RVSP) value, TRV value, aortic root dimensions, PFT. METHODS Cobb angle, 2D-echo signs of structural heart disease, aortic root dimensions, tricuspid regurgitant jet velocity (TRV), PFTs, ABG, and patient demographics were reviewed. Right ventricular systolic pressure (RVSP) was estimated using Bernoulli's equation [4(TRV)2] and right atrial pressure. RVSP ≥ 36mmHg is a surrogate marker for pulmonary hypertension. Logistic regression was used to assess for differences in TRV between groups. RESULTS Mean preoperative RVSP was significantly elevated in AIS patients (26.9±0.49; p CONCLUSIONS This study found that 21% of patients with AIS had elevated TRV while age-matched controls had no TRV abnormalities. Additionally, RVSP measurements demonstrated mild pulmonary hypertension in AIS patients. These abnormal values normalized postoperatively, indicating the benefits of scoliosis surgery on cardiac function in AIS. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.

Pulmonary hypertension with adult respiratory distress syndrome: prevalence, clinical impact, and association with central venous pressure.

Pulmonary hypertension (PH) occurs in patients with acute respiratory distress syndrome (ARDS); the most severe form comprises acute cor pulmonale (ACP). Here, we investigated the prevalence of PH in patients with ARDS to evaluate its correlation with ACP risk, ARDS severity and central venous pressure (CVP). We conducted a secondary analysis using data from the MIMIC-III open-source clinical database. The prevalence of PH associated with new-onset ARDS during the first 72 h after intensive care unit admission was investigated; moreover, the association between ACP risk score and PH was validated. We also evaluated the association between elevated CVP (mean CVP > 10 mmHg) and PH and other clinical outcomes. Among 2434 patients who met the ARDS Berlin criteria and underwent echocardiography or pulmonary artery catheterization evaluation, a total of 583 (24.0%) patients were diagnosed with moderate or severe PH, of which 418 had low and 165 had high ACP risk. After adjustment for disease/ARDS severity, ACP risk score, and other demographic variables, elevated CVP was independently associated with the occurrence of PH (odds ratio, 2.239 (1.674, 2.993), p < 0.005). Among patients with PH, higher mean CVP was associated with prolonged hospital stay (13.4 vs. 15.2 days, p = 0.041) and duration of ventilation (116.5 vs. 150.5 h, p = 0.023). Incidence of PH was 24.0% in patients with new-onset ARDS in this retrospective study. Elevated CVP is relevant with higher incidence of PH and worse clinical outcome; these highlighted the importance of hemodynamic monitoring in the management of ARDS.

Significant functional mitral regurgitation affects left atrial function in heart failure patients: haemodynamic correlations and prognostic implications.

Functional mitral regurgitation (FMR) is a well-known pathophysiological factor in heart failure (HF) patients, and left atrial function (LAF) is a novel determinant of clinical status and outcome in this setting. However, little is known about the pathophysiological role of FMR on LAF in HFrEF patients. Aim of this study is to explore the possible interplay between the severity of FMR and LAF in heart failure with reduce ejection fraction (HFrEF) patients and their possible consequences. We studied 97 consecutive patients with FMR classified in two groups: mild-to-moderate MR ore less (FMR group, n = 38) and moderate-to-severe or more (SFMR group, n = 59). Using the phasic method, left atrial contractile, conduit, reservoir, and total emptying function (TLAEF) were calculated to assess LAF. SFMR group showed significantly lower values of LAF compared to FMR group. LA dysfunction (LA-dys) was defined for TLAEF values below the median and groups divided in four subgroups based on its presence. Patient with LA-Dys in SFMR group showed a worse clinical status, higher incidence of right ventricular dysfunction (RV-Dys), and pulmonary hypertension (PH), and a significant worse clinical survival compared to all other groups. In our study, the survival was significantly lower in SFMR/LA-Dys+ group. Furthermore, LA-Dys was strongly related with worse clinical status and higher incidence of PH and RV-Dys. These results suggest that in patients with SFMR and HFrEF, LA-Dys may represent both a marker of more advanced disease and a novel prognostic factor.

Racial differences in patients referred for right heart catheterization and risk of pulmonary hypertension.

African Americans (AA) have a higher incidence of pulmonary hypertension (PH) risk factors. Few studies have examined the racial differences in the prevalence and etiology of PH and direct comparison of invasive hemodynamics between AAs and Caucasians has rarely been reported. In this study, we examined whether racial differences exist in patients referred for right heart catheterization (RHC) and hypothesized that AA race is an independent risk factor for PH and is associated with increased adjusted mortality. We extracted data for AA and Caucasian patients who underwent RHC at Vanderbilt between 1998 and 2014. Clinical information was obtained from Vanderbilt's Synthetic Derivative, a de-identified mirror of our Electronic Medical Record. A total of 4576 patients were analyzed, including 586 (13%) AAs and 3990 (87%) Caucasians. AAs were younger than Caucasians by an average of eight years, but had more prevalent heart failure, features of metabolic syndrome, and higher creatinine. AAs also had higher mean pulmonary artery pressure and pulmonary vascular resistance. After adjusting for relevant co-morbidities, the AA race is associated with 41% increased risk of PH (odds ratio [OR] = 1.41, 95% confidence interval [CI] = 1.12–1.79). Among patients with PH, AA race is associated with 24% increased adjusted mortality (hazard ratio [HR] = 1.24, 95% CI = 1.09–1.45). AAs were younger but had more prevalent cardiometabolic and renal disease and worse pulmonary hemodynamics. The AA race is an independent risk factor for PH. Among patients with PH, the AA race is associated with increased adjusted mortality. Future studies should focus on delineating whether genetic or environmental factors contribute to PH risk in AAs.

Distinct patterns of response to transfusion therapy for different chronic complications of sickle cell disease: A useful insight

Two main sub-phenotypes have been described in sickle cell disease: one with higher baseline haemoglobin and a higher rate of painful crises and one with lower baseline haemoglobin, increased markers of haemolysis and a higher incidence of pulmonary hypertension, priapism and leg ulcers. We compared the patterns of response to regular automated red cell exchange transfusion over a five-year period of 21 patients with recurrent painful crises from the first group and 3 patients with pulmonary hypertension and 5 with recurrent severe stuttering priapism form the second and found them to be distinctly different. Response for pain is slow and increases gradually over years. The most pronounced clinical benefit and the one that appears first is a reduction in the severity rather than the frequency of painful crises. In contrast to the slow and gradual response we see for pain, response of patients with pulmonary hypertension and priapism is immediate with significant clinical improvement even after the first transfusion. The response appears to be directly correlated to the HbS level as the symptoms of both conditions invariably recur rapidly when transfusions are delayed or discontinued but resolve again once they are re-instituted.

Congenital heart diseases in women

Are there gender differences in prevalence, surgical results and long-term survival of patients with congenital heart disease? Available literature data allow us to state what follows. At birth there is a mild but significant prevalence of congenital heart disease in females. The most severe congenital heart diseases are less frequent in girls, but when they are present in females, they are linked to a higher surgical mortality rate, due perhaps to lower weight at birth and to the prevalence of extracardiac malformations and/or of associated genetic syndromes. On the other hand, in adults, surgery for congenital heart disease is at higher risk in males, and so the long-term survival rate is higher in females. Particular psychological attitudes, a higher incidence of pulmonary hypertension, as well as specific problems linked to the reproductive function characterize congenital heart disease in adult women. The knowledge and analysis of these data are essential for a correct management of congenital heart disease in neonates, children and adults.

Risk factors for pulmonary hypertension in patients receiving maintenance peritoneal dialysis.

We investigated the risk factors for pulmonary hypertension (PH) in patients receiving maintenance peritoneal dialysis (MPD). A group of 180 end-stage renal disease patients (124 men and 56 women; mean age: 56.43±8.36) were enrolled in our study, which was conducted between January 2009 and June 2014. All of the patients received MPD treatment in the Dialysis Center of the Second Affiliated Hospital of Soochow University. Clinical data, laboratory indices, and echocardiographic data from these patients were collected, and follow-ups were scheduled bi-monthly. The incidence and relevant risk factors of PH were analyzed. The differences in measurement data were compared by t-test and enumeration data were compared with the χ2 test. Among the 180 patients receiving MPD, 60 were diagnosed with PH. The remaining 120 were regarded as the non-PH group. Significant differences were observed in the clinical data, laboratory indices, and echocardiographic data between the PH and non-PH patients (all P<0.05). Furthermore, hypertensive nephropathy patients on MPD showed a significantly higher incidence of PH compared with non-hypertensive nephropathy patients (P<0.05). Logistic regression analysis showed that the proportion of internal arteriovenous fistula, C-reactive protein levels, and ejection fraction were the highest risk factors for PH in patients receiving MPD. Our study shows that there is a high incidence of PH in patients receiving MPD and hypertensive nephropathy patients have an increased susceptibility to PH.

Surviving Birth at Any Altitude

In the 16th century, Potosi (3976 m) was one of the most important cities in the world, because of its silver mountain. A Spaniard chronicle reported 100% mortality of newborns from Spaniard parents at that time, and women migrated to lower altitude cities to give birth. Several generations later, “the miracle of Saint Nicolas” occurred and a boy survived in Potosí after 6 dead siblings. Since that time, more and more children from sea level mothers (not high altitude natives) survive birth at high altitude, and the genes for postnatal adaptation to chronic hypobaric hypoxia are widespread in the 21st century. Some factors—such as birth weight, maternal anemia, pre-eclampsia, and existence of congenital diseases—have been clearly defined as prognostic for surviving birth. Pregnancy at high altitude is associated with a reduction in birth weight of approximately 100 g per 1000 m of ascent. Low birth weight affects mostly children from mothers without high altitude ancestry, but not native babies, and evidence of endoplasmatic reticulum stress and protein synthesis inhibition in the placenta of nonnative women at high altitude have been found. Children from natives may be protected by the augmented uterine artery flow described for this group. Also, there is the possibility that elevated enzymatic antioxidant activity contributes to the Andean protection against altitude-associated small for gestational age infants. Hemoglobin concentrations in mothers at high altitude are well studied. When appropriate adjustments were used, maternal anemia was associated with lower infant Apgar scores at both 1 minute and 5 minutes after birth, as well as complication of labor, lower gestational age at birth, and higher parity. Little is reported about congenital heart diseases, their natural history, especially regarding pulmonary hypertension, and the unique physiology of indigenous high altitude residents in this context. Our investigation team has been working on this last topic since 2003. We report the results of 4000 echocardiographic studies performed in 3500 patients born at 3600 m in La Paz, Bolivia, not older than 3 months. The patient group was selected by pediatricians and referred because of heart murmur, cyanosis, syndromal fenotype, heart failure, oxygen dependency, and enhanced cardiac size in the Rx. Of these, 20.1% of the children showed no abnormal finding, 18.1% showed pulmonary hypertension (defined as systolic pulmonary pressure of more than 40 mm Hg or more than two thirds from the systemic pressure) in the absence of congenital heart disease, 14.6% showed ventricular septal defect, 20% presented with patent ductus arteriosus, 14.2% atrial septal defect, 5.1% tricuspidal atresia, 2.6% complete atrioventricular channel, 2.5% tetralogy of Fallot, 2.5% Shone complex, and 0.4% others. We assume that the high incidence of pulmonary hypertension in this group, with oxygen dependency and right ventricle hypertrophy for weeks, is due to a prolonged postnatal adaptation period. The most important reason for this phenomenon is the late remodeling of the pulmonary artery branches, which implies the persistence of a thick musculary media. The persistent elevation of the pulmonary pressure and hypoxia are predisposing factors to patency of the ductus arteriosus. Right-sided congenital heart diseases like tricuspid atresia are 3 times more frequent in our patient cohort than at sea level; being left-sided significantly lowers their incidence. Environmental and genetic circumstances may both play a role in the pathogenesis of congenital heart disease at high altitude.

Why there is a need to discuss pulmonary hypertension other than pulmonary arterial hypertension?

Pulmonary hypertension (PH) is a condition characterized by the elevation of the mean pulmonary artery pressure above 25 mmHg and the pulmonary vascular resistance above 3 wood units. Pulmonary arterial hypertension (PAH) is an uncommon condition with severe morbidity and mortality, needing early recognition and appropriate and specific treatment. PH is frequently associated with hypoxemia, mainly chronic obstructive pulmonary disease and DPLD and/or left heart diseases (LHD), mainly heart failure with reduced or preserved ejection fraction. Although in the majority of patients with PH the cause is not PAH, a significant number of published studies are still in regard to group I PH, leading to a logical assumption that PH due to other causes is not such an important issue. So, is there a reason to discuss PH other than PAH? Chronic lung diseases, mainly chronic obstructive lung disease and DPLD, are associated with a high incidence of PH which is linked to exercise limitations and a worse prognosis. Although pathophysiological studies suggest that specific PAH therapy may benefit such patients, the results presented from small studies in regard to the safety and effectiveness of the specific PAH therapy are discouraging. PH is a common complication of left heart disease and is related to disease severity, especially in patients with reduced ejection fraction. There are two types of PH related to LHD based on diastolic pressure difference (DPD, defined as diastolic pulmonary artery pressure - mean PAWP): Isolated post-capillary PH, defined as PAWP > 15 mmHg and DPD < 7 mmHg, and combined post-capillary PH and pre-capillary PH, defined as PAWP > 15 mmHg and DPD ≥ 7 mmHg. The potential use of PAH therapies in patients with PH related to left heart disease is based on a logical pathobiological rationale. In patients with heart failure, endothelial dysfunction has been proposed as a cause of PH and hence as a target for treatment, supported by the presence of increased endothelin-1 activity and impaired nitric oxide-dependent vasodilation. Unfortunately, so far, there is no evidence supporting the use of specific PAH therapies in patients with PH related to left heart disease. In conclusion, the presence of PH in patients with conditions other than PAH contributes to the severity of the disease, affecting the outcome and quality of life. The disappointing results regarding the effectiveness of specific PAH therapies in patients with chronic lung diseases and LHD underline the need for seeking new underlying mechanisms and thus novel therapies targeting PH due to left heart disease and/or lung diseases.

Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation

IntroductionPatients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. MethodsRecords of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed. Results285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%–60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses. ConclusionGiven equivalent lung volumes (40%–60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome.

Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center.

Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE. We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La. Two-step clustering and Kaplan-Meier survival analyses were used. Five clusters were identified. A cluster consisted of patients with only anti-dsDNA antibodies, a cluster of anti-Sm and anti-RNP, a cluster of aCL IgG/M and LAC, and a cluster of anti-Ro and anti-La antibodies. Analysis revealed 1 more cluster that consisted of patients who did not belong to any of the clusters formed by antibodies chosen for cluster analysis. Sm/RNP cluster had significantly higher incidence of pulmonary hypertension and Raynaud phenomenon. DsDNA cluster had the highest incidence of renal involvement. In the aCL/LAC cluster, there were significantly more patients with neuropsychiatric involvement, antiphospholipid syndrome, autoimmune hemolytic anemia, and thrombocytopenia. According to the Systemic Lupus International Collaborating Clinics damage index, the highest frequency of damage was in the aCL/LAC cluster. Comparison of 10 and 20 years survival showed reduced survival in the aCL/LAC cluster. This study supports the existence of autoantibody clusters with distinct clinical features in SLE and shows that forming clinical subsets according to autoantibody clusters may be useful in predicting the outcome of the disease. Autoantibody clusters in SLE may exhibit differences according to the clinical setting or population.

Left ventricular surgical remodelling: is it a matter of shape or volume?.

Left ventricular surgical remodelling (LVSR) can be targeted to volume reduction (VR), (independently of the final shape) or to conical shape (CS). The aim of this study was to evaluate the long-term clinical and echocardiographic results of these two surgical strategies. From January 1988 to December 2012, 401 patients underwent LVSR: 107 in Group VR (1988-2001) and 294 in Group CS (1998-2012). The latter group of patients had lower ejection fraction (EF) and higher mitral and tricuspid regurgitation grade, with higher incidence of pulmonary hypertension. A propensity score model was built to adjust long-term results for preoperative and operative profiles. Thirty-day mortality was 6.0%. Median follow-up interval time was 100 (3-300) months. Overall 20-year and event-free survival were 36.1 ± 7.8 and 19.4 ± 7.2, respectively. No differences were found regarding 10-year survival (Group VR: 55.1 ± 4.8 vs Group CS: 64.2 ± 4.2, P = 0.16) and event-free survival (Group VR: 41.1 ± 4.8 vs Group CS: 50.5 ± 4.8, P = 0.12). However, Group CS provided better 10-year freedom from cardiac deaths (74.5 ± 3.7 vs 60.4 ± 4.8, P = 0.03) and from cardiac events (55.6 ± 5.0 vs 45.0 ± 4.9, P = 0.04). After propensity score adjustment, all the main outcomes were significantly better in Group CS. Multivariate Cox analysis confirmed this result; furthermore, to avoid any bias related to improved experience, 30-day mortality being higher in Group VR, we excluded the first month from Cox analysis: left ventricle VR (independently of the final shape) was still confirmed as the wrong approach. At the follow-up, Group CS showed significant improvement in EF (+18 vs +8%), end-systolic volume index (-35 vs -20%) and sphericity index (-6 vs +9%). LVSR should aim to provide a more physiological shape (conical) rather than simple VR.