Pulmonary Artery Calcification Research Articles

Attenuating effect of magnesium on pulmonary arterial calcification in rodent models of pulmonary hypertension.

Vascular calcification has been considered as a potential therapeutic target in pulmonary hypertension. Mg2+ has a protective role against calcification. This study aimed to investigate whether Mg2+ could alleviate pulmonary hypertension by reducing medial calcification of pulmonary arteries. Monocrotaline (MCT)-induced and chronic hypoxia-induced pulmonary hypertension rats were given an oral administration of 10% MgSO4 (10 ml/kg per day). Additionally, we administered Mg2+ in calcified pulmonary artery smooth muscle cells (PASMCs) after incubating with β-glycerophosphate (β-GP, 10 mmol/l). In vivo, MCT-induced and chronic hypoxia-induced pulmonary hypertension indexes, including right ventricular systolic pressure, right ventricular mass index, and arterial wall thickness, as well as Alizarin Red S (ARS) staining-visualized calcium deposition, high calcium levels, and osteochondrogenic differentiation in pulmonary arteries, were mitigated by dietary Mg2+ intake. In vitro, β-GP-induced calcium-rich deposits stained by ARS, calcium content, as well as the detrimental effects of calcification to proliferation, migration, and resistance to apoptosis of PASMCs were alleviated by high Mg2+ but exacerbated by low Mg2+. Expression levels of mRNA and protein of β-GP-induced osteochondrogenic markers, RUNX Family Transcription Factor 2, and Msh Homeobox 2 were decreased by high Mg2+ but increased by low Mg2+; however, Mg2+ did not affect β-GP-induced expression of SRY-Box Transcription Factor 9. Moreover, mRNA expression and protein levels of β-GP-reduced calcification inhibitor, Matrix GLA protein was increased by high Mg2+ but decreased by low Mg2+. Mg2+ supplement is a powerful strategy to treat pulmonary hypertension by mitigating pulmonary arterial calcification as the calcification triggered physiological and pathological changes to PASMCs.

A STICKY SITUATION

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Patients with cirrhosis regularly undergo endoscopy for management of portal hypertensive complications. Little is known about the pulmonary complications of such procedures. Here, we present an acute pulmonary complication post gastric variceal gluing. CASE PRESENTATION: A 61-year-old female with type 2 diabetes, hypertension, and Child Pugh A cirrhosis secondary to non-alcoholic steatohepatitis presented with a two-day history of right-sided shoulder and sub-diaphragmatic pain, 3 days after elective injection of a gastric varix with Glubran and Lipidol. She described the pain as being sharp and pleuritic. She denied shortness of breath, hemoptysis, or constitutional symptoms. She had a temperature of 36.7 degrees Celsius, heart rate of 83 beats/min, blood pressure of 136/81, respiratory rate of 18/min, and an oxygen saturation of 91% on room air. Her physical exam was unremarkable. She was tender to palpation of the right sub-diaphragmatic area. Stigmata of chronic liver disease were not noted. Her investigations revealed normal electrolytes, creatinine, hemoglobin and white blood cell count. Her platelets were 111, INR 1.2, ALT 27 U/L, Bilirubin 38 umol/L, GGT 43 U/L, and the D-dimer was 1.82 mg/L. Her chest x-ray showed several new nodular densities in both lungs. Computed tomography pulmonary angiogram reported diffuse pulmonary arterial calcification; it was later addended to reveal a large caliber variceal gastrorenal shunt and high density filling defects consistent with severe burden of embolized glue throughout the segmental and subsegmental pulmonary vasculature bilaterally. DISCUSSION: The use of glue is commonly used in endoscopic therapeutics to prevent and treat gastrointestinal bleeding [1,2]. It has an associated 0.5%-4.3% risk of embolization [1], which can result in cardiopulmonary collapse [2]. Embolization is believed to occur through travel through gastrorenal and splenorenal veins into the inferior vena cava, with eventual deposition into the pulmonary circulation [2]. Large, high-flow portosystemic shunts and glue injections of more than 1 mL are predicted risk factors for pulmonary embolization [3]. The differential diagnosis for our patient included septic emboli, pulmonary embolus, malignancy, or pulmonary hypertension associated with vessel calcification. Initial management included administration of Cefazolin for a possible infection, followed by watchful waiting as the patient was hemodynamically stable. A secondary review of her imaging by Radiology further confirmed the findings of embolized glue. CONCLUSIONS: Pulmonary embolization of glue is an uncommon endoscopic complication of variceal treatment that has been linked to complications such as cardiopulmonary collapse and septic emboli [2]. It is important that clinicians keep this on the differential diagnosis when evaluating patients with chest pain or hemodynamic instability after gluing of varices. Reference #1: Al-Hillawi L, Wong T, Tritto G, Berry PA. Pitfalls in histoacryl glue injection therapy for oesophageal, gastric and ectopic varices: A review. World J Gastrointest Surg. 2016;8(11):729–734. doi:10.4240/wjgs.v8.i11.729 Reference #2: Hameed F, Pepperell J, Sidney J. A rare complication of endoscopic intervention. Breathe. 2018;14(2):e40-e42. doi:10.1183/20734735.019017 Reference #3: Jindal A, Philips CA. Pulmonary Glue Embolization after Endoscopic Treatment of Bleeding Sigmoid Colon Varices. ACG Case Rep J. 2017;4:e52. Published 2017 Apr 12. doi:10.14309/crj.2017.52 DISCLOSURES: No relevant relationships by Jennifer Landry, source=Web Response No relevant relationships by Dana Saleh, source=Web Response

Pulmonary Artery Calcification in Eisenmenger Syndrome and Pulmonary Arterial Hypertension (ES-PAH): Correlation with Clinical Outcomes

Introduction: Pulmonary artery (PA) calcification is a well-documented radiological finding in patients with severe Pulmonary Arterial Hypertension (PAH) and is most common in Eisenmenger Syndrome (ES). The aim of this study was to evaluate the prognostic implications of PA calcification in the ES cohort.

Granzyme B deficiency promotes osteoblastic differentiation and calcification of vascular smooth muscle cells in hypoxic pulmonary hypertension

Calcification is a major risk factor for vascular integrity. This pathological symptom and the underlying mechanisms in hypoxic pulmonary artery hypertension remain elusive. Here we report that pulmonary vascular medial calcification is elevated in pulmonary artery hypertension models as a result of an osteoblastic phenotype change of pulmonary arterial smooth muscle cells induced by hypoxia. Notably, inhibiting store-operated calcium channels significantly decreased osteoblastic differentiation and calcification of pulmonary arterial smooth muscle cells under hypoxia. We identified granzyme B, a major constituent of cytotoxic T lymphocytes/natural killer cell granules involved in apoptosis, as the main regulator of pulmonary arterial calcification. Overexpression of granzyme B blocked the mineralization through its effect on store-operated calcium channels in cultured pulmonary arterial smooth muscle cells under hypoxic conditions. Mice with overexpression of granzyme B exposed to hypoxia for 3 weeks showed attenuated vascular calcification and pathological progression of hypoxic pulmonary arterial hypertension. Our findings emphasize the central function of granzyme B in coordinating vascular calcification in hypoxic pulmonary arterial hypertension.

Development of a technique for determination of pulmonary artery pulse wave velocity in horses.

Calcification of the tunica media of the axial pulmonary arteries (PA) has been reported in a large proportion of racehorses. In humans, medial calcification is a significant cause of arterial stiffening and is implicated in the pathogenesis of cardiac, cerebral, and renal microvascular diseases. Pulse wave velocity (PWV) provides a measure of arterial stiffness. This study aimed to develop a technique to determine PA-PWV in horses and, secondarily, to investigate a potential association between PA-PWV and arterial fibro-calcification. A dual-pressure sensor catheter (PSC) was placed in the main PA of 10 sedated horses. The pressure waves were used to determine PWV along the PA, using the statistical phase offset method. Histological analysis of the PA was performed to investigate the presence of fibro-calcified lesions. The mean (±SD) PWV was 2.3 ± 0.7 m/s in the proximal PA trunk and 1.1 ± 0.1 m/s further distal (15 cm) in a main PA branch. The mean (±SD) of mean arterial pressures in the proximal PA trunk was 30.1 ± 5.2 mmHg, and 22.0 ± 6.0 mmHg further distal (15 cm) within the main PA branch. The mean (±SD) pulse pressure in the proximal PA trunk was 15.0 ± 4.7 mmHg, and 13.5 ± 3.3 mmHg further distal (15 cm) within the main PA branch. Moderate to severe lesions of the tunica media of the PAs were observed in seven horses, but a correlation with PWV could not be established yet. Pulmonary artery PWV may be determined in standing horses. The technique described may allow further investigation of the effect of calcification of large PAs in the pathogenesis of equine pulmonary circulatory disorders.NEW & NOTEWORTHY Pulmonary artery pulse wave velocity was determined safely in standing sedated horses. The technique described may allow further investigation of the effect of calcification of large pulmonary arteries in the pathogenesis of pulmonary circulatory disorders in horses.

Abstract 14631: Pulmonary Artery Calcification: A New Threat in Pulmonary Arterial Hypertension

Background: Pulmonary arterial hypertension (PAH) is associated with imbalance between proliferation and apoptosis of pulmonary artery (PA) smooth muscle cells (PASMCs). Recently, we uncovered a role for microRNA-204 (miR-204), whose downregulation sustains the phenotype in time. In systemic vascular disease, miR-204 downregulation increases the Runt-related transcription factor 2 (RUNX2), a key osteogenic factor inducing calcification. The fact that PAH patients live longer increases the odds of developing PA calcification lesions. Interestingly, the presence and development of calcified lesions in PAH have not been explored. We thus hypothesized that miR-204-dependent upregulation of RUNX2 triggers the development of proliferative and calcified vascular lesions within the lungs, thus worsening PAH. Methods/Results: Using CT-scan, we showed that PAH patients (n=50; p<0.05) had significant increase in distal PA calcification compared to control patients matched for age, sex and comorbidities known to promote vascular calcification. This non-invasive finding was histologically confirmed in the same patients post lung transplantation using Von Kossa staining (n=10; p<0.05). Finally, increase in calcification contributes to worsening of PA compliance and is associated with poorer PAH patients’ survival (n=50; p<0.05). At the molecular level, enhanced calcification in PAH patients correlates (n=43; p<0.05) with a miR-204-dependent up regulation of RUNX2 in lungs, distal PA and primary cultured PASMCs (n=12-40, fold change ≈ 2.5). Using gain and loss of function approaches, we demonstrated in PASMCs from PAH and healthy donors (using siRNA and adenoviruses respectively) that RUNX2, promotes PAH-PASMC proliferation (Ki67), resistance to apoptosis (Annexin-V) and calcification (Alizarin red) (p<0.05). Finally, in Sugen/hypoxia rat model of PAH, we demonstrated that RUNX2 inhibition improves pulmonary hemodynamic and reduces PA remodeling and calcification (n=5-12, p<0.05). Conclusion: We demonstrated for the first time the presence of significant calcification in PAH distal PA contributing to PAH worsening and identified RUNX2 as an attractive new therapeutic strategy to improve PAH. *SC and GR equally contributed.

CT features of pulmonary arterial hypertension and its major subtypes: a systematic CT evaluation of 292 patients from the ASPIRE Registry

We evaluated the prevalence and prognostic value of CT-pulmonary angiographic (CTPA) measures in 292 treatment naive patients with pulmonary arterial hypertension (PAH). Pulmonary artery calcification (13%) and thrombus (10%) were...

Intermedin/adrenomedullin2: an autocrine/paracrine factor in vascular homeostasis and disease.

Intermedin (IMD) or adrenomedullin 2 is a novel peptide related to the calcitonin gene-related peptide (CGRP) family. Via calcitonin receptor-like receptor/receptor activity modifying proteins, the common receptor complexes of CGRP, IMD exerts a wide range of biological effects, especially regulation of cardiovascular homeostasis. Proteolytic processing of a larger IMD precursor yields a series of biologically active C-terminal fragments, IMD1-53, IMD1-47 and IMD8-47. IMD and its receptors are present in the cardiovascular system, and IMD is present at low levels in plasma. In the cardiovascular system, IMD has multiple functions such as regulation of blood pressure and cardiac function, pro-angiogenesis, endothelial barrier function protection, anti-oxidative stress, and anti-endoplasmic reticulum stress. IMD participates widely in the pathogenesis of atherosclerosis, hypertension, pulmonary arterial hypertension and vascular calcification. It is a vascular regulatory factor of homeostasis and a vital endogenous protective factor against vascular diseases.

Implication of miR‐204/RUNX2‐dependent calcification pathway in pulmonary arterial hypertension (1089.23)

Introduction: Pulmonary arterial hypertension (PAH) is a vascular remodeling disease characterized by occurrence of several vascular lesions. Interestingly, some biological pathways implicated in these lesions are also implicated in calcification processes. Nonetheless, the role for calcification lesions in PAH has never been explored. miR‐204, which is downregulated in PAH, is implicated in calcification processes by directly regulating the pro‐calcification transcription factor RUNX2. RUNX2 also regulates cell proliferation by activating HIF‐1. Thus, we hypothesized that miR‐204 downregulation in PAH triggers RUNX2 expression promoting calcification and vascular lesions in PAH. Methods and results: Using human explanted lungs, fresh distal pulmonary arteries (PAs) and freshly isolated pulmonary artery smooth muscle cells (PASMC) from both control and PAH patients, we demonstrated a significant downregulation of miR‐204 and an upregulation of RUNX2 in PAH compared to controls. The increase in RUNX2 in PAH patients was associated with an increase in distal PA calcification. Gain‐ and loss‐of‐function approaches in human PASMC showed that downregulation of miR‐204 in control PASMC increases RUNX2 expression, while increased miR‐204 in PAH‐PASMC decrease it. More importantly, restoring miR‐204 or decreasing RUNX2 expression in PAH‐PASMC decreases proliferation, resistance to apoptosis and alkaline phosphatase activity. Finally, in Sugen/hypoxia PAH rat model, nebulization of either miR‐204 mimic or RUNX2 siRNA significantly improves PAH.Conclusion: Taken together, our study uncovers a new miR‐204/RUNX2 axis contributing to both calcified lesions and vascular remodeling in distal PA of PAH patients. More experiments are required to further explore the role of RUNX2 in PAH pulmonary artery calcification. This study might lead to the discovery of new potential therapeutic strategies for patients suffering of PAH.

A case of isolated bilateral pulmonary arterial calcification diagnosed in utero

We report a very rare case of isolated multiple pulmonary arterial calcification with severe bilateral peripheral pulmonary arterial stenosis diagnosed in utero. Despite treatment with bisphosphonate for 6 months, systolic right ventricular pressure increased persistently and surpassed left ventricular pressure. After successful bilateral pulmonary arterioplasty at 13 months of age, the patient showed decreased systolic right ventricular pressure with normal interventricular septal configuration. This is the first case report for an isolated pulmonary artery calcification without other arterial calcification proven by non-contrast computed tomography of a living patient.

Pulmonary valve, pulmonary artery and right ventricular calcification in a case of chronic pulmonary thromboembolism

Calcification of pulmonary valve and pulmonary artery in association with large, organized, calcified right ventricular thrombi is unusual. Herein we describe a case of 40 year male with history of recurrent pulmonary embolism with right ventricle, pulmonary valve and a pulmonary artery calcification. The role of non-invasive studies in the diagnosis and long term follow up of patient is emphasized.

Pulmonary Artery Calcification in Contact with a Left Coronary Artery Bypass Graft: An X-ray Computed Tomographic Study.

Calcification in the pulmonary artery (PA) occurs in rare cases. There have been no studies of calcification in the PA at the site of its contact with a left coronary artery bypass graft (CABG). In the present study, X-ray computed tomography (CT) was employed for examination of such calcification. The subjects were 53 patients (49 male and 4 female, mean age of 56.7 years) who underwent 74 left CABGs (69 saphenous veins and five internal thoracic arteries). Following surgery, non-contrasted CT was performed from the lower level of the aortic arch to the lower boundary of the left ventricle at 5-mm horizontal intervals, and contrasted CT was performed at the level of the PA; this procedure was repeated at approximately six-month intervals after the operation. In addition, aortography and selective graft angiography were carried out at 7.6 months postoperatively. The inner diameter of the grafts and the levels of serum cholesterol were also examined. Calcification in the PA was detected in 24 cases (all of them saphenous vein grafts), but graft angiography found no stenosis in those sites. Calcification size varied from 1 mm to 14 mm, with 10 of the cases at or exceeding 10 mm and showing high density. Only three of the cases enlarged with time. Calcification appeared at 2.9 to 54.3 months postoperatively and the mean time of onset was 10.0 +/- 15.7 months. The mean age of the patients with PA calcification was 58.7 +/- 5.9 years while that of the patients without calcification was 57.3 +/- 10.0 years. Graft diameter was 5.9 +/- 1.9 mm in the former group and 5.6 +/- 1.7 mm in the latter. Serum cholesterol level was 235 +/- 32 mg/dl in the former group and 243 +/- 42 mg/dl in the latter. There were three cases of occlusion in the calcification group, and four in the other. There were no significant intergroup differences in these four parameters. The incidence of CT-detected calcification in the PA was found to be high at its point of contact with saphenous vein grafts. </hea

Pulmonary Artery Calcification in Racehorses

Pulmonary Artery Calcification in Racehorses

Pulmonary artery calcification in racehorses may be related to transient and repeated increases in arterial pressure during exercise

Calcification of the pulmonary artery has been found in a large number of racing horses. The majority of calcified lesions are found immediately distal to the primary arterial bifurcation. Increased arterial wall stress levels have been previously demonstrated at these locations, with the wall stress levels increasing under intra-luminal pressures associated with exercise. We hypothesize therefore that the formation of calcified lesions is mediated by transient and repeated increases in pulmonary artery intra-luminal pressure. The presence of calcified lesions would likely further exacerbate the levels of wall stress, leading to growth of the lesions. A level of wall stress may exist above which calcified lesions form, and a second level may exist above which the calcified lesions grow at an increased rate. A computer model of pulmonary artery wall stress with calcified lesions was created, and wall stress levels were found to be greatest at the periphery of the calcified lesions. Osteo/chondrocyte-like cells have also been found at the periphery of the calcified lesions and could be responsible for collagen deposition and lesion growth, mediated by local wall stress levels. These increased levels of wall stress could place racehorses at a greater risk of acute pulmonary arterial rupture at the site of the calcified lesions, due to the high levels of intra-luminal pressure within the pulmonary artery during exercise. The hypothesis may also have implications in the etiology of human vascular diseases.

Metastatic Pulmonary Calcification in Renal Failure Mimicking Pulmonary Embolism on Lung Scan

Metastatic pulmonary calcification (MPC) characterized by diffuse calcium deposition in the lungs is known to occur in patients with chronic renal failure. However, MPC with pulmonary artery calcification is uncommon and has only been detected in a few patients with severe disorders. A 48-year-old man with chronic renal failure had cough and progressive dyspnea. Ventilation-perfusion (V/Q) lung scans showed multiple large-sized mismatched V/Q defects in the left middle and lower zones of lungs, which was consistent with a high probability of pulmonary embolism (PE). The findings of pulmonary scintigraphy resulted from MPC with pulmonary artery calcification, revealed by simultaneous technetium-99m MDP scintigraphy, low-dose computed tomography, and high-resolution computed tomography (HRCT) of the chest.

The diagnosis of patent ductus arteriosus with Eisenmenger's syndrome by three-dimensional computed tomography: A case report

The diagnosis of patent ductus arteriosus (PDA) with left-to-right shunt can be made on the basis of transthoracic echocardiographic examination [1]. However, the diagnosis of PDA with right-to-left shunt by using transthoracic echocardiography is difficult because of the lack of typical color flow associated with PDA [2]. In this report, we describe the utility of 3-dimensional computed tomography (CT) to demonstrate presence of PDA in a patient with suspected PDA with Eissenmenger’s syndrome. A 47-year-old woman was admitted to our department with dyspnea on exertion for 32 years. Patient’s vital signs were normal. On physical examination, an intense second heart sound on the pulmonary area and a grade 3/6 pansystolic murmur on the pulmonary, tricuspid and mesocardiac areas were auscultated. The patient’s jugular veins were distended at approximately 15 cm H2O. Both her feet and left hand were cyanotic, and lower extremities had bilateral 1+ pitting edema. The electrocardiogram showed right axis, right bundle branch block, right ventricular hypertrophy and biatrial enlargement. The chest radiography demonstrated moderate cardiomegaly, prominent pulmonary arterial conus and calcification between aortic and pulmonary arch. Transthoracic echocardiogram revealed right ventricular hypertrophy and dilatation, pulmonary arterial and biatrial dilatation, elevated pulmonary arterial pressure (systolic pressure was 120 mmHg). No congenital cardiac abnormalities were seen. According to finger pulse

Pulmonary arterial and intracranial calcification in the recipient of a twin–twin transfusion

Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin-twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.

Pulmonary artery calcification in contact with a left coronary artery bypass graft: An X-ray computed tomographic study

Calcification in the pulmonary artery (PA) occurs in rare cases. There have been no studies of calcification in the PA at the site of its contact with a left coronary artery bypass graft (CABG). In the present study, X-ray computed tomography (CT) was employed for examination of such calcification. The subjects were 53 patients (49 male and 4 female, mean age of 56.7 years) who underwent 74 left CABGs (69 saphenous veins and five internal thoracic arteries). Following surgery, non-contrasted CT was performed from the lower level of the aortic arch to the lower boundary of the left ventricle at 5-mm horizontal intervals, and contrasted CT was performed at the level of the PA; this procedure was repeated at approximately six-month intervals after the operation. In addition, aortography and selective graft angiography were carried out at 7.6 months post-operatively. The inner diameter of the grafts and the levels of serum cholesterol were also examined. Calcification in the PA was detected in 24 cases (all of them saphenous vein grafts), but graft angiography found no stenosis in those sites. Calcification size varied from 1 mm to 14 mm, with 10 of the cases at or exceeding 10 mm and showing high density. Only three of the cases enlarged with time. Calcification appeared at 2.9 to 54.3 months postoperatively and the mean time of onset was 10.0 ± 15.7 months. The mean age of the patients with PA calcification was 58.7 ± 5.9 years while that of the patients without calcification was 57.3 ± 10.0 years. Graft diameter was 5.9 ± 1.9 mm in the former group and 5.6 ± 1.7 mm in the latter. Serum cholesterol level was 235 ± 32 mg/dl in the former group and 243 ± 42 mg/dl in the latter. There were three cases of occlusion in the calcification group, and four in the other. There were no significant intergroup differences in these four parameters. The incidence of CT-detected calcification in the PA was found to be high at its point of contact with saphenous vein grafts.

Pulmonary Artery Calcification in Contact with a Left Coronary Artery Bypass Graft: An X-ray Computed Tomographic Study

Calcification in the pulmonary artery (PA) occurs in rare cases. There have been no studies of calcification in the PA at the site of its contact with a left coronary artery bypass graft (CABG). In the present study, X-ray computed tomography (CT) was employed for examination of such calcification. The subjects were 53 patients (49 male and 4 female, mean age of 56.7 years) who underwent 74 left CABGs (69 saphenous veins and five internal thoracic arteries). Following surgery, non-contrasted CT was performed from the lower level of the aortic arch to the lower boundary of the left ventricle at 5-mm horizontal intervals, and contrasted CT was performed at the level of the PA; this procedure was repeated at approximately six-month intervals after the operation. In addition, aortography and selective graft angiography were carried out at 7.6 months post-operatively. The inner diameter of the grafts and the levels of serum cholesterol were also examined. Calcification in the PA was detected in 24 cases (all of them saphenous vein grafts), but graft angiography found no stenosis in those sites. Calcification size varied from 1 mm to 14 mm, with 10 of the cases at or exceeding 10 mm and showing high density. Only three of the cases enlarged with time. Calcification appeared at 2.9 to 54.3 months postoperatively and the mean time of onset was 10.0 ± 15.7 months. The mean age of the patients with PA calcification was 58.7 ± 5.9 years while that of the patients without calcification was 57.3 ± 10.0 years. Graft diameter was 5.9 ± 1.9 mm in the former group and 5.6 ± 1.7 mm in the latter. Serum cholesterol level was 235 ± 32 mg/dl in the former group and 243 ± 42 mg/dl in the latter. There were three cases of occlusion in the calcification group, and four in the other. There were no significant intergroup differences in these four parameters. The incidence of CT-detected calcification in the PA was found to be high at its point of contact with saphenous vein grafts.

Eisenmenger syndrome in adults: Ventricular septal defect, truncus arteriosus, univentricular heart

OBJECTIVESMorbidity and mortality patterns were characterized in adults with the Eisenmenger syndrome when two ventricles with a ventricular septal defect (VSD) joined two great arteries or one great artery, or when one ventricle joined two great arteries.BACKGROUNDAlthough afterload in these disorders differs, clinical differences have not been defined.METHODSSeventy-seven patients were studied. Group A comprised 47 patients with VSD, aged 23 to 69 years (mean 39.5 ± 10.2), follow-up 5 to 18 years (mean 7.2 ± 4.9); group B, 14 patients with truncus arteriosus, aged 27 to 50 years (mean 33.7 ± 7.3), follow-up 6 to 18 years (mean 7.7 ± 5.1), and group C, 16 patients with univentricular heart, aged 18 to 44 years (mean 30.6 ± 8.4), follow-up 5 to 15 years (mean 4.4 ± 4.2). Echocardiography established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, noncardiac surgery and the multisystem disorders of cyanotic adults.RESULTSThirty-five percent of the patients died. Sixty-three percent of deaths were sudden, and resulted from intrapulmonary hemorrhage, rupture of either the pulmonary trunk, ascending aorta or a bronchial artery, or vasospastic cerebral infarction, or the cause was unestablished. There were no documented tachyarrhythmic sudden deaths.CONCLUSIONSMedical management of coexisting cardiac disease, multisystem systemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurysms, thromboses and calcification; to truncal valve stenosis and regurgitation; to semilunar and atrioventricular valve regurgitation, and to major risks of nontachyarrhythmic sudden death.