Dilated Pulmonary Artery Research Articles

Role of chest radiographs and electrocardiograms in predicting the hemodynamics of congenital heart disease

Aim: This study aimed to evaluate the role of chest radiographs and electrocardiograms in predicting the hemodynamics of congenital heart disease (CHD). Methods: This retrospective study included 50 patients with a diagnosis of CHD who had undergone any form of cardiac intervention, either surgical or nonsurgical between September 2019 and September 2020. Chest radiographs and electrocardiograms were evaluated and compared with the diagnostic gold standard echocardiography. Results: Chest radiographs had the highest sensitivity, specificity, and accuracy, with all being 100%, in detecting situs and cardiac position. There was a very good agreement between chest radiographs and echocardiography in the detection of both situs and cardiac position (κ = 1.00, P < 0.001), while moderate agreement was observed for the detection of cardiomegaly, position of the aortic knuckle, main pulmonary artery dilation, and right pulmonary artery dilation. Electrocardiograms had a high sensitivity (100.00%), but modest specificity and accuracy for the detection of left ventricle pressure overload. For the detection of left atrial enlargement and left ventricle volume overload, electrocardiograms had high specificity (94.12% and 94.29%, respectively) but low sensitivity and modest accuracy. There was a moderate agreement between electrocardiograms and echocardiography in the detection of right ventricle pressure overload (κ = 0.43, P = 0.002) and left ventricle volume overload (κ = 0.46, P < 0.001). Conclusions: The study findings indicate that chest radiographs and electrocardiograms alone are not adequate for the assessment of hemodynamics of CHD and reinstates the recommendation that in addition to routine chest radiographs and electrocardiograms, echocardiography should be performed.

Pulmonary Artery Aneurysm Associated with Sarcoidosis in a 75-year-old with Heart Failure: The Challenges of Diagnosis and Management.

Pulmonary artery aneurysm (PAA) is a rare abnormality of pulmonary vasculature. It can be idiopathic or secondary to various pathologies, frequently with multiple factors leading to its formation. We report the case of a man with concomitant sarcoidosis and PAA. A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis. PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure. Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.

Analysis of echocardiographic features and postnatal management of fetal pulmonary valve stenosis

Objective: To investigate the echocardiographic features, consistency of diagnosis between fetal and postnatal periods and postnatal clinical outcomes of fetal pulmonary valve stenosis (PS) with different degrees. Methods: This study was a retrospective cohort study comprising 108 cases of fetal PS diagnosed during the fetal period and followed up postnatally at Xinhua Hospital, Shanghai Jiaotong University School of Medicine from November 2012 to February 2023. Echocardiographic characteristics, including morphological and hemodynamic features were collected for all fetuses who were then were followed up to at least 6 months after birth. One-way analysis of variance and Kruskal-Wallis test were used to compare the differences in the echocardiographic features among fetuses with different degrees of PS. Subsequently, McNemar test was used to assess the consistency of diagnosis between the fetal and postnatal periods. Furthermore, Logistic regression analysis was applied to explore the risk factors for neonatal intervention in fetuses with moderate PS and the receiver operating characteristic (ROC) curve was utilized to ascertain the optimal cut-off value for continuous variables. Results: The age of the mothers of the 108 fetuses at the initial assessment was (30.8±4.0) years, and the gestational age was 26.5 (24.6, 30.0) weeks. The fetuses were categorized into mild (17 cases), moderate (49 cases), and severe groups (42 cases) based on the initial echocardiographic features. Mild PS was characterized by valve thickening and hyperechogenicity combined with systolic flow acceleration or dilation of main pulmonary artery. Moderate PS exhibited both restricted valve motion and a colorful blood flow pattern at the valve orifice. The peak flow velocities of fetuses with moderate and critical PS were notably higher than those in the mild group ((2.66±0.86) and (2.77±1.30) vs. (1.43±0.59)m/s, F=14.52, P<0.001). In critical PS, all cases showed retrograde ductal flow, with a significantly higher proportion of a small right ventricle compared to the mild and moderate PS (42.9% (18/42) vs. 0 and 2.0% (1/49), χ2=31.73, P<0.001). The proportion of severe tricuspid regurgitation was also higher (35.7% (15/42) vs. 0 and 10.2% (5/49), χ2=36.94, P<0.001). Compared to mild and severe PS, the consistency of diagnosis between fetal and postnatal periods in moderate PS was lower (40.8% (20/49) vs.13/17 and 80.3% (35/42), χ2=12.45, P=0.006). The systolic flow velocity was identified as an independent risk factor for neonatal intervention in fetuses with moderate PS (OR=7.21, 95%CI2.11-24.62). A flow velocity of ≥2.18 m/s in second trimester and ≥3.15 m/s in third trimester indicated the necessity of neonatal intervention for fetal moderate PS. Among the 108 fetuses, 68 underwent surgical intervention and all survived. Additionally, 39 fetuses were regularly followed up. A sole non-surgical fatality occurred, leading to a 6-month survival rate of 99.1% (107/108). Conclusions: Various degrees of fetal PS demonstrate distinctive morphological and hemodynamic alterations in echocardiography. The disparity in severity between the postnatal and fetal stages requires ongoing monitoring for fetal PS. The prognosis for fetal PS is generally favorable.

Prevalence of pulmonary artery dilation in non-cystic fibrosis bronchiectasis: a computed tomography analysis from a cohort of the US Bronchiectasis and Nontuberculous Mycobacteria Research Registry.

Although pulmonary artery (PA) dilation is independently associated with significant morbidity and mortality in patients with pulmonary diseases irrespective of diagnosed pulmonary hypertension, its relationship with nontuberculous mycobacteria (NTM) is unknown. The Bronchiectasis and NTM Research Registry is a multicenter registry created to foster research in non-cystic fibrosis (CF) bronchiectasis and NTM lung disease. The majority of patients with non-CF bronchiectasis at Oregon Health & Science University have NTM infections. To determine the prevalence of PA dilation in these patients and its association with supplemental oxygen use, severity of bronchiectasis, tobacco use, and NTM in the sputum culture, we evaluated the chest computed tomography (CT) scans from 321 patients in a cross-sectional analysis. We measured the severity of bronchiectasis by applying modified Reiff criteria and measured the diameters of the PA and aorta (Ao), with PA dilation defined as a PA:Ao ratio >0.9. In our cohort, the mean age was 67.3 years and 83.2% were female. The mean modified Reiff score was 7.1, indicating moderate disease severity. Forty-two patients (13.1%) were found to have PA dilation. PA dilation was positively associated with the use of supplemental oxygen (P<0.001), but there was no association between PA dilation and NTM infection.

Silenced by heart – Ortner’s syndrome (cardio-vocal hoarseness) – An unusual manifestation of a distant dreadful disease: A cross-sectional observational study

ABSTRACT Aims and Objectives: This study aims to explore the pathogenesis of hoarseness of voice resulting from cardiovascular disorders that affect the left recurrent laryngeal nerve (LRLN), supported by a review of the existing literature. Materials and Methods: This is a cross-sectional observational study conducted over a 3-year period, spanning from May 2019 to May 2022. The study involved the evaluation of 720 cases of hoarseness of voice through video-laryngoscopy. Within this cohort, we scrutinized eight distinct cases of Ortner’s syndrome, each attributed to various underlying causes. All patients with LRLN paralysis underwent a comprehensive clinical assessment, which encompassed chest X-rays, echocardiography, and chest computed tomography scans. Results: Out of the eight Ortner’s syndrome cases, six were associated with dilated pulmonary arteries, stemming from various etiologies such as rheumatic heart disease, mitral valve prolapse, pulmonary embolism, interstitial lung disease, portopulmonary hypertension, and patent ductus arteriosus. One case presented with pericardial effusion and pulmonary hypertension, while another case featured a dilated aorta secondary to severe aortic regurgitation. Among these cases, three patients underwent valve replacement, one underwent pericardiocentesis, and the remaining cases were managed conservatively. Only one case that underwent mitral valve replacement showed partial improvement in voice, while there was no significant improvement in the other cases. All cases remained under follow-up. Conclusion: Although cardiovascular-related hoarseness is an unusual presentation, it should be kept in mind for all cases of altered voice with underlying cardiac disease. Indirect laryngoscopy should be routinely performed in all cases of cardiac illness having hoarseness of voice.

Evaluation of prebiotic supplementation on lipid peroxidation and hematologic parameters of broilers with ascites

Evaluation of prebiotic supplementation on lipid peroxidation and hematologic parameters of broilers with ascites

Case Report of Absent Pulmonary Valve with Intact Interventricular Septum

Abstract Absent pulmonary valve syndrome is a rare cardiac malformation, usually associated with tetralogy of Fallot. Its presence with intact interventricular septum is even less common with case reports or very small series in the literature. We describe a case who presented with symptoms of right heart failure due to an absent pulmonary valve with severe pulmonary regurgitation and small atrial septal defect. Diagnostic evaluation revealed grossly dilated main pulmonary artery and rudimentary leaflets of the pulmonary valve with severe regurgitation. Ventricular septum was intact and there was no patent ductus arteriosus. Later, the patient underwent surgical prosthetic pulmonary valve implantation.

Plasmodium Falciparum Malaria and Concomitant High-Risk Pulmonary Thromboembolism: Case Report

The authors describe a case of a patient admitted due to a previous condition of osteomyoarthralgia, headache, fever, malaise, which worsened with sudden onset dyspnea, chest pain, syncope, the plasmodium test was positive, the EKG performed at the Emergency department presented the classic pattern of acute cor pulmonale (S1Q3 T3 ). The echocardiogram demonstrated changes compatible with pulmonary thromboembolism. Chest X-ray showed an increase in the cardiothoracic index and dilation of the right pulmonary artery (Fleischner ‘s sign). Computed angiotomography (angioCT) of the chest confirmed the diagnosis, showing the presence of thrombi in the main, segmental and subsegmental branches. The patient underwent fibrinolytic therapy and progressed favorably.

Abstract 11597: A Rare Cause of an NSTEMI: Left Main Coronary Artery Compression in a Patient With Pulmonary Hypertension

Background: Pulmonary hypertension has varying effects on cardiac function. Though uncommon, a dilated pulmonary artery can cause decreased coronary perfusion through extrinsic compression of the left main coronary artery (LMCA). While the most common presentation of LMCA compression is angina, acute coronary syndrome is rare. Case Summary: An 81-year-old female with a history of group 3 pulmonary hypertension presenting with neck and shoulder pain was found to have elevated cardiac biomarkers and EKG findings remarkable for isolated ST elevation in aVR with diffuse ST depressions in all other leads, consistent with a non-ST elevation myocardial infarction. Coronary angiography revealed left main coronary artery compression that was successfully intervened on with placement of a single drug eluding stent. Right heart catheterization was remarkable for evidence of severe pulmonary hypertension with a mean pulmonary artery pressure of 60 mmHg. Discussion: Given the patient’s past medical history, presenting symptoms, coronary and right heart catheterization findings the etiology of her acute coronary syndrome presentation was thought to be driven by left main coronary artery compression from pulmonary artery dilation. Pulmonary artery dilation in individuals with a history of pulmonary hypertension is common, with several modalities available to evaluate for this anatomic compensatory measure. Management for left main coronary artery compression in this patient population is percutaneous coronary intervention. Future studies to determine if routine screening for coronary compression in patients with pulmonary hypertension is needed.

Relationship of Main Pulmonary Artery (Truncus Pulmonalis) Diameter With Hospital Stay and Mortality in Pulmonary Hypertension Patients Admitted to the Emergency Department.

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological disease significantly associated with morbidity and mortality. The increase in pulmonary vascular resistance, high pulmonary artery pressure and wall tension that occurs in PH results in dilatation of the main pulmonary artery (truncus pulmonalis), one of the largest and most important vessels in the body. The aim of this study is to investigate the relationship between the diameter of the truncus pulmonalis and hospitalization, length of hospital stay, and mortality in patients diagnosed with PH. Demographic characteristics, number of Emergency Department (ED) admissions, post-admission status, treatment, truncus pulmonalis diameter, and mortality were evaluated statistically through the patient files of 115 PH patients who presented to the ED of Fırat University Faculty of Medicine,Elazığ, Türkiye, between January 2022 and December 2022. Of the 115 PH patients who came to the ED, 70 (60.8%) were women and 45 (39.2%) were men, with a mean age of 78.77±8.72 years. Fifty-one of these patients were discharged from the ED after treatment, and 64 were hospitalized. The mean length of hospital stay was two (min=0, max=38) days. Thoracic CT scans demonstrated that the mean diameter of the truncus pulmonalis of the patients was 34.874±3.288 mm (35.20±3.6509 mm in women, 34.367±2.5836 mm in men; p₌0.351) and there was no statistically significant relationship with mortality (p=0.496), hospitalization (p=0.806), and length of hospital stay (p=0.416). There was a statistically significant relationship between mortality rate and male gender (p=0.02) and comorbidity (p=0.001). It was determined that there was no statistically significant relationship between the truncus pulmonalis diameter and gender, comorbidity, hospitalization, length of hospital stay, and mortality in this study in which single-centre one-year admissions of PH, which differ in aetiology, epidemiology, and demographic features were examined. However, among the patient demographics, a significant relationship was determined between gender and the number of comorbidities and mortality.

Pulmonary artery dilatation in different causes of pulmonary hypertension.

Pulmonary artery (PA) dilatation is commonly observed in patients with pulmonary hypertension (PH). However, the clinical aspects of PA dilatation in various etiology of PH remain unknown. In this study, we investigated the clinical and imaging characteristics of 1018 patients with different subtypes of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The independent determinants for all-cause death were identified using univariate and multivariate Cox proportional hazard models. PA dilatation was identified in 88.8% of the patients, and 27.2% had a PA diameter/ascending aorta diameter ratio greater than 1.5. PA diameter was shown to be significantly correlated with hemodynamic parameters and symptom duration in idiopathic PAH patients. PA diameter only correlated with pulmonary circulation volume in patients with PAH associated with congenital heart disease. PA diameter correlated with symptom duration and right ventricular end-diastolic dimension in CTEPH patients. PA diameter correlated with right ventricular end-diastolic dimension in patients with PAH associated with connective tissue disease. Only 6-min walk distance, but not PA dilatation, predicts all-cause death independently. In conclusion, PA dilatation is a common finding in PH patients. The clinical feature of PA dilatation varies greatly between PH types. PA dilatation is not associated with all-cause death.

Role of right heart catheterization in diagnosis of pulmonary arterial hypertension

Abstract Background Pulmonary hypertension (PH) presumably has an impact on 1% or less of the world’s population. Right Heart Catheterization (RHC) is essential for determining PH problems. RHC determines prognosis and offers helpful information on the degree of hemodynamic impairment. Therefore, this comparative study was carried out to determine the diagnostic role, pitfalls associated, and complications encountered during RHC in suspected cases of PAH and chronic thromboembolic pulmonary hypertension (CTEPH). Methods The observational cohort study was conducted on 75 patients with clinical suspicion of PH and CTEPH. All patients were subjected to full history taking, ECG, Echocardiography, and radiological investigations including Chest radiography (CXR), high-resolution computed tomography (HRCT), and computed tomography (CT) pulmonary angiography. Results The mean pulmonary artery mean pressure (PAMP) was 46.05 ± 9.631 mmHg, the mean Pulmonary artery dilatation (PAD) was 30.04 ± 1.082 mm the mean ejection fraction (EF%) was 63.87%, the mean right ventricular diameter was 45.83 ± 0.444 mm. The mean pulmonary artery wedge pressure (PAWP) was 10.91 ± 2.303 mmHg, and the mean cardiac output (CO) was 4.00 ± 0.773 L/min mean cardiac index (CI) was 1.94 ± 0.416 L/min/m2. Doppler echocardiographic estimated pulmonary artery systolic pressure (DE-ePASP) and estimated pulmonary, artery, end-diastolic pressure (ePADP) significantly positively correlated with RHC-PASP (P &lt; 0.001) and mean DE-ePAP significantly positively Correlated with mean RHC-PAP (P &lt; 0.001). Conclusions RHC is still the only method that can completely and accurately diagnose PAH. however, the combination between right cardiac catheterization and echocardiography provides a more accurate diagnosis of the diseases and their complications.

Simultaneous Double Balloon Dilatation for Supravalvar Pulmonary Obstruction After Arterial Switch Operation.

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7-3.8) years; 9.8(8.1-15.1) kgs underwent the procedure at 14(8-44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12-14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9months, IQR 7-22, range 5-73, others are free of re interventions with median RVOT gradient of 42, IQR 27-49, range 21-55mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results.

Radiographic findings of cardiopulmonary structures can predict hypertrophic cardiomyopathy and congestive heart failure in cats.

This study aimed to find the radiographic characteristics for the detection of hypertrophic cardiomyopathy (HCM) and congestive heart failure (CHF) in cats. Healthy cats (n = 35) and HCM cats with (21) and without (22) CHF. On radiography, the cardiac size using vertebral heart score, left atrium enlargement (LAE), and dilation of the pulmonary vessels were assessed. The sensitivity and specificity of the radiographic characteristics regarding LAE were evaluated with the echocardiographic left atrium to aortic root ratio as a reference. In HCM cats, cardiomegaly, LAE, and dilation of the caudal pulmonary artery were found compared with those in healthy cats. The LAE could be predicted using the elevation of the carina with 94.12% specificity but 17.5% sensitivity. When CHF developed, LAE and dilation of the caudal pulmonary vein (PV) were significantly different compared with those in HCM cats without CHF. The distal side of the summated shadow made by the right caudal PV with the ninth rib in HCM cats with CHF was significantly larger than that in HCM cats without CHF and a cut-off value of 5.35 mm was drawn with 75% sensitivity and 100% specificity. Although there was an overlapping of radiographic findings between healthy and HCM cats, radiographic assessment of LAE can be useful for predicting HCM and the distal side of the summated shadow made by the right caudal PV with the ninth rib can predict CHF in HCM cats.

Sleep Apnea Combined with Pulmonary Hypertension in a Veteran Patient Population.

We have investigated the concurrence of sleep apnea and pulmonary hypertension in a Veteran population. We retrospectively reviewed 142 patients who underwent chest CT scans and had a dilated main pulmonary artery, defined as a width exceeding 29 mm on axial images. Approximately 40% of patients with pulmonary hypertension had associated sleep apnea. No significant difference in pulmonary artery diameters could be found between the group without sleep apnea and the group with sleep apnea (34.5 ± 4.2 mm vs. 34.7 ± 4.4 mm, p = 0.373).

Multislice computed tomography angiography in assessment of pulmonary vascular abnormalities

Abstract Background Multidetector computed tomography (CT) angiography with maximum intensity projection, multiplanar reformations and 3D reconstructions, and volume rendering technique provide visualization of normal vascular anatomy and variants. Aim The aim of this work was to evaluate the role of multislice CT angiography in the evaluation of pulmonary vascular diseases, classification of diseases, and to be familiar with technique and finding. Patients and methods This observational trial was carried out on 30 cases suspected of or diagnosed with acute chest pain, suspected pulmonary embolism, and pulmonary arterial or pulmonary venous abnormalities. Patients were classified equally into two groups. All patients underwent pulmonary angiography using CT, which involved the following steps: preparation of the patient, examination procedure, data collection, reconstruction and postprocessing of the images, and image explanation. Results In group 1, 60% of cases had normal pulmonary vein whereas 20% of them had venous drainage anomaly. The majority of cases (86.7%) had confluent pulmonary artery findings. The highest percent of cases had dilatation of main pulmonary artery, left pulmonary artery (46.7%), and right pulmonary artery. Overall, 26.7% had either atretic or hypoplastic main pulmonary artery, 40% had normal right pulmonary artery, and 26.7% had normal left pulmonary artery. In group 2, 80% of cases had normal pulmonary vein, whereas 20% had abnormal findings. The majority of cases (80.0%) had pulmonary embolism, and 33.3% had pulmonary hypertension (one case had both embolism and hypertension). Conclusions CT angiography is a very effective and beneficial method in the evaluation of pulmonary vascular abnormalities with or without congenital cardiac and/or extracardiac anomalies.

Off-label use of KONAR-MF™ occluder for transcatheter closure of patent ductus arteriosus in unusual circumstances.

Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations. Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5-41) and 10 kg (5-98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5-18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg). KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

P406 LAUBRY–PEZZI SYNDROME: EVERYTHING IS NOT AS IT SEEMS

Abstract Introduction Laubry–Pezzi syndrome is a congenital heart disease characterized by the prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD), mostly supracristal, leading to severe aortic regurgitation (AR). It has been reported nearly always in childhood, the Venturi effect is the predominant factor and onset symptoms may be variable. Case Report A 61–years–old male was admitted to our intensive case unit for chest pain radiated to interscapular region and exertional dyspnea. Hemodynamic parameters were normal. At ECG sinus rytm with heart rate 92 bpm and non–specific repolarization abnomalities were reported. Hs troponin I was 276 ng/L, myoglobin 30.2 ng/mL and BNP 325 pg/mL. Transthoracic echocardiography showed a dilated left ventricle (LV EDV 107 mL/m2) with normal ejection fraction (55%), severe AR without aortic root dilation and a suspicious shunt left to right at level of interventricular septum. Therefore, transesophageal echocardiography was performed and confirmed severe AR by probable bicuspid valve and right cusp prolapse, in absence of calcifications and/or vegetations by endocarditis, restrictive perimembranous VSD (maximum diameter 0.6 cm) and ostium secundum ASD with shunt L–R. Right chambers were not dilated and non invasive systolic pulmonary artery pressure was 43 mmHg. Coronary angiography was performed and showed a significant stenosis of distal left anterior descending (LAD) artery and of proximal circumflex artery. Right heart catetherization excluded significant high values of pulmonary pressures (mPAP=18 mmHg), of pulmonary capillary wedge pressure (9 mmHg), with a mild increase of pulmonary vascular resistances (1.16 HRU; nv &amp;lt; 96). Coronary artery bypass grafting (left internal mammary artery on LAD and Y–graft radial artery on obtuse marginal artery), aortic valve replacement with bioprothesis (Inspiris Resilia 23 mm), ASD closure by direct suture and VSD closure with pericardial patch were performed. No post–surgical complications were reported. Interestingly, during the replacement of aortic valve, it resulted tricuspid, confirming the suspicion of Laubry Pezzi syndrome. Conclusions our case was object of great debate because restrictive VSD and severe AR were not previously known, were diagnosed in middle age and until then paucisymptomatic. Laubry–Pezzi syndrome is a very rare disease that may explain controversial cases of severe AR.

P394 PULMONARY HYPERTENSION IN SEARCH OF GROUP

Abstract Episode of haemoptysis in a 25–year–old woman with the following medical history: surgical closure of Botallo‘s duct at the age of 5 months, coeliac disease, growth retardation, polycystic ovary, visus alterations. In the emergency room CT angiography of the chest showed the presence of subpleural parenchymal consolidation in the right lower lobe, with associated ground–glass opacities, cardiomegaly, severe pulmonary artery dilatation and hepatomegaly with intrathoracic involvement by a hernia of Morgagni on the right side. A porto–systemic shunt with an intrahepatic course was also noted in the duct at the VIII segment with an autonomic outlet in the right atrium. The patient was transferred to the Internal Medicine ward. Objective examination revealed an elf facies with hypertelorism, short stature, bradypsychism. On interview she reported dyspnoea on light exertion and frequent lipohymic episodes. Haematochemical examinations showed increased of bilirubinemia mainly indirect, Transaminases and of NTproBNP and normal ammonium levels. Blood gas analysis showed a tendency to respiratory alkalosis with oxyaemia at lower limits. Because of the suspicion of a syndromic picture, a genetic examination was performed. Cardiological evaluation showed a high ultrasound probability of pulmonary hypertension (PH). Transferred to cardiology, catheterisation confirmed pulmonary arterial hypertension (mPAP 64mmHg) precapillary (wedge 4mmHg) with high PVR (11 Wood Units) not responsive to nitric oxide. In addition, there was a superior vena cava protruding into the coronary sinus and an extrahepatic shunt with an isolated outlet in the RA without gradient. The hepatobiliary surgery consultation concluded for Abernethy syndrome, which is a rare congenital syndrome characterised by an extrahepatic portosystemic shunt in which the blood from the splanchnic and lienal circulation bypasses the portal vein and the liver, draining into the systemic circulation via an abnormal conduit. PH is a rare complication of this syndrome, maintained by hyperafflux, vasoactive mediators and the formation of microthrombi and intimal fibrosis in the pulmonary circulation. An HR–CT excluded venulo–capillary plexiform disease. Macitentan 10 mg/day was introduced, then continued at home for the benefit shown and follow–up at the Pulmonary Work Group was initiated. This PH, due to its association with an extremely rare pathology, required the collaboration of a diverse group of specialists.

Long-Term Survival of Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Single-Center Cohort.

Pulmonary arterial hypertension (PAH) is a rare but severe complication of connective tissue disease (CTD). CTD-associated PAH (CTD-PAH) is the most common subgroup of PAH in East Asia. We prospectively collected 41 patients with CTD-PAH and followed them for a mean period of 43 ± 36 months. The long-term survival rates of the CTD-PAH patients at 1, 2, 3 and 5 years were 90%, 80%, 77%, and 60%, respectively. The non-survivors had more dilated main pulmonary arteries, higher pulmonary artery pressure and pulmonary vascular resistance (PVR). PAH-specific therapy resulted in improvements in functional class, 6-minute walk distance, serum uric acid, right ventricular function and PVR. Increased C-reactive protein during follow-up, indicating inflammatory processes, was also crucial for the management of CTD-PAH. Therefore targeting both PAH and inflammation is important in this specific subgroup of PAH. The results of this study may help develop treatment strategies for CTD-PAH patients.