Dilated Main Pulmonary Artery Research Articles

Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR.

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 &lt; 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 &lt; 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.

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.

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).

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.

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.

MASSIVE PULMONARY ARTERY ANEURYSM: AN UNCOMMON CAUSE OF MYOCARDIAL ISCHEMIA

MASSIVE PULMONARY ARTERY ANEURYSM: AN UNCOMMON CAUSE OF MYOCARDIAL ISCHEMIA

Eisenmenger syndrome with left main compression syndrome: a case report

BackgroundLeft main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH.Case presentationA 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis.ConclusionsLeft main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.

EP188: Novel HECW2 variant presenting with tachypnea and multisystemic congenital malformations

HECW2, otherwise known as NEDL2, encodes for a member of the HECT family of E3 ubiquitin ligases and stabilizes p73, a key mediator in neurodevelopment. HECW2 is primarily expressed in the brain, heart, and lungs. Heterozygous variants have been associated with developmental delays, intellectual disability, epilepsy, hypotonia, scoliosis and dysmorphic features. Systemic complications which have been reported include cardiomyopathy, skeletal abnormalities including pectus excavatum, and various brain abnormalities including white matter disease, cortical atrophy, ex-vacuo ventriculomegaly, and thinning of the corpus callosum. While many reported cases of HECW2-related disorder have been from de novo variants, there have been reports of familial inheritance with variable expressivity. To date, there have been no reported cases of HECW2-related disorder presenting with persistent tachypnea. A newborn male with a history of intrauterine growth restriction born at 39 2/7 weeks gestation to a 31 year old G4P0212 mother and a 35 year old father presented in the perinatal period with unexplained persistent tachypnea, difficulty feeding, persistent diaphoresis and failure to thrive. Pectus excavatum was noted at birth and initial evaluation for the persistent tachypnea showed multiple vertebral anomalies, congenital scoliosis and mild cardiomegaly on chest X-ray. Renal ultrasound demonstrated left central calyceal dilation. Echocardiogram and CT heart with contrast demonstrated multiple abnormalities including anomalous pulmonary venous return with right upper pulmonary vein draining into the superior vena cava (SVC), secundum atrial septal defect (ASD), dilated main pulmonary artery, right atrium and ventricle, mild right and left ventricular hypertrophy, mild tricuspid regurgitation and mild to moderate pulmonary regurgitation. CT imaging also demonstrated severe tracheobronchomalacia associated with left mainstem bronchus compression resulting in air trapping within the left lung. Cardiac catheterization was attempted for possible closure of the patient’s secundum ASD, but was unsuccessful due to inability to capture retroaortic rim. Diagnostic evaluation during catheterization demonstrated mild pulmonary hypertension, normal pulmonary vascular resistance (PVR) and a Qp:Qs of 1.9:1 consistent with pulmonary overcirculation. Tachypnea and significantly increased work of breathing persisted despite initiation of furosemide diuresis to manage pulmonary overcirculation. The patient ultimately required BiPap for respiratory support, though tachypnea persisted. A G-tube was placed to ensure safe formula administration. Additional physical exam findings included hypotonia, macrocephaly, infraorbital creases with almond-shaped eyes, ocular hypertelorism, broad nasal bridge and tip, mild retrognathia, arachnodactyly with larger hands and feet compared to growth centiles and Y-shaped gluteal fold. Family history was remarkable for White ancestry. A maternal half-sister was healthy. A 2 year old brother had speech delay. The patient’s father had a history of mild congenital scoliosis, heart murmur and intellectual disability. The patient’s mother had a history of anemia, heart murmur and learning delays. There was no history of genetic conditions, recurrent pregnancy loss, or infant death. Chromosomal microarray analysis (CMA) demonstrated no clinically significant findings (ISCN: arr[hg19](1-22x2,(X,Y)x1). Trio exome sequencing with mitochondrial analysis, performed from a peripheral blood sample, demonstrated a paternally inherited likely pathogenic variant in HECW2 (NM_020760.1:c.2581 C>T) in exon 11. While this variant has not been previously published as benign or pathogenic, in silico analysis demonstrated a potential deleterious effect of the arginine to tryptophan substitution on structure and/or function. Mitochondrial analysis was negative/normal. We present the first documented case of HECW2-related disorder presenting with perinatal tachypnea. The patient’s hypotonia, feeding difficulties, dysmorphic features, pectus excavatum, vertebral malformations, scoliosis, and cardiac involvement correlate with previously described manifestations of HECW2-related disorder.

PULMONARY ARTERY ANEURYSM- IDIOPATHIC

SUMMARY&#x0D; A 28-years old female presented with complaints of palpitations and mild retrosternal chest pain. CXR showed a prominent Pulmonary artery. A TTE study revealed a huge main pulmonary artery whereas the branches were of normal size. CT angio revealed aneurysmal main Pulmonary artery and normal sized branches. No concomitant etiology was noted, hence labelled as “idiopathic pulmonary artery aneurysm”.&#x0D; CASE DESCRIPTION&#x0D; A 28-year-old female presented with retrosternal chest pain and palpitations for the last six months. Multiple systolic clicks and murmur III/VI heard in the pulmonary area. ECG was unremarkable. Chest X-Ray showed no cardiomegaly and a prominent pulmonary artery. Transthoracic echocardiography showed aneurysmally dilated main pulmonary artery (size: 5.7cm). Pulmonary valve was thickened with mild regurgitation. CT angiogram demonstrated dilated main pulmonary artery (axial dimension: 5.8cm) with normal-sized branches (Figure1). Immunological workup was negative. She was advised surgical intervention which she agreed to pursue soon at a tertiary care hospital near her hometown.&#x0D; LEARNING POINTS&#x0D; &#x0D; Pulmonary artery aneurysm has an incidence of 1 in 14,000, (based on 109,571 autopsies).1&#x0D; The maximum normal diameter of MPA is 29mm in males and 27 mm in females. For interlobar branches the maximum size it 17 mm.2 If the size exceeds 4 cm it is “aneurysmal” which may be true of false.&#x0D; PAA could be congenital due to Eisenmenger’ syndrome, Pulmonary valve stenosis and absent pulmonary valve syndrome. Connective tissue disorders (Marfan’s syndrome, alpha-1 antitrypsin deficiency and Ehlers-Danlos syndrome) and autoimmune disorders (Behcet’s disease and Hughes-Stovin syndrome).3&#x0D; Acquired causes include PAH, auto-immune disease (vasculitis), trauma infections, malignancy and idiopathic.&#x0D; For intervention no consensus of opinion is available. However expert opinion recommends it when the patient becomes symptomatic or pulmonary artery diameter exceeds 5 cm to 6 cm.&#x0D; &#x0D; QUESTIONS WITH ANSWERS&#x0D; &#x0D; Question 1: Is there any difference in the measurement of PA size by echocardiography and CT?&#x0D; Question 2: What is the usual site of dissection in PAA?&#x0D; Question 3: In Pulmonary arterial tree what is the most frequent site of aneurysm?&#x0D; Question 4: Some cases of pulmonary artery aneurysms can be managed medically- Y/N?&#x0D; Question 5: The technique of choice for diagnosing PA aneurysm is pulmonary angiography or CT angiogram?&#x0D; &#x0D; Answers&#x0D; &#x0D; Question 1: Yes&#x0D; Question 2: Main PA&#x0D; Question 3: Right lobar artery&#x0D; Question 4: Yes&#x0D; Question 5: CT angiogram&#x0D; &#x0D; References&#x0D; &#x0D; Deterling RA Jr and Clagett OT. Aneurysm of the pulmonary artery; review of the literature and report of a case. Am Heart J. 1947;34:471-99.&#x0D; Truong QA, Massaro JM, Rogers IS, Mahabadi AA, Kriegel MF, Fox CS. Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: The Framingham Heart Study. Circ Cardiovasc Imaging 2012;5:147-54.&#x0D; Agarwal S, Chowdhury UK, Saxena A, Ray R, Sharma S, Airan B. Isolated idiopathic pulmonary artery aneurysm. Asian Cardiovasc Thorac Ann. 2002;10(2):167-9.&#x0D;

Epidemiological study of acute pulmonary embolism in a tertiary care center

Background: Acute pulmonary embolism remains a significant cause of morbidity and mortality. This study aimed to determine the clinical profile, management, and outcomes of patients with acute pulmonary embolism. Methods: This was a retrospective, single-center, and observational study. All consecutive patients with proven diagnosis of pulmonary embolism and treated at a tertiary care center were included in the study based on inclusion and exclusion criteria. Results: From January 2012 to May 2018, a total of 304 patients diagnosed with pulmonary embolism were included in the study. Majority of the patients (n = 195; 64.14%) were male. Among included patients, 92 (30.66%) were smokers, and 81 (26.6%) were obese. Dyspnea (98.03%) was the most commonly observed symptom. Of patients who underwent bilateral lower limb venous Doppler, deep vein thrombosis was noted in 172 (56.57%). Electrocardiography confirmed the presence of sinus tachycardia and classical S1Q3T3 pattern in 284 (93.42%) and 79 (25.99%) patients, respectively. Chest X-ray showed dilated main and right pulmonary arteries in 170 (55.92%) patients. Right ventricular dysfunction as detected by echocardiography was observed in 241 (79.28%) patients. Thrombolytic therapy was administered in 158 (51.97%) patients (n = 86 (28.29%) were treated with tenecteplase; n = 72 (23.68%) were treated with streptokinase). Conclusions: This study gives an insight into the clinical profile of patients hospitalized with a confirmed diagnosis of acute pulmonary embolism along with treatment and diagnostic approaches adopted by the physician in the Indian scenario.

Berry syndrome: a case report and literature review

BackgroundBerry syndrome, a rare combination of cardiac anomalies, consists of aortopulmonary window (APW); aortic origin of the right pulmonary artery; interrupted aortic arch (IAA) or hypoplastic aortic arch or coarctation of the aorta; and an intact ventricular septum. There is lack of review articles that elucidate the clinical features, diagnosis, treatment, and outcomes of Berry syndrome. This publication systematically reviews the 89 cases published since 1982 on Berry syndrome.Case presentationA 38-year-old woman presented with a loud murmur and cyanosis. Transthoracic echocardiography demonstrated a severely dilated aorta and main pulmonary artery with a large intervening defect. Distal to the APW, the ascending aorta gave rise to the right pulmonary artery. Additionally, a type A IAA, an intact ventricular septum, and a large patent ductus arteriosus were revealed. Computed tomography angiography with 3-dimensional reconstruction confirmed above findings. This is the first report of a patient of this age with Berry syndrome who did not undergo surgery.ConclusionsBerry syndrome is a rare but well-identified and surgically correctable anomaly. Patients with Berry syndrome should be followed up for longer periods to better characterize long-term outcomes.

Abstract 14310: Elephant Sitting on My Chest: A Tale of Two Vessels

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Unfavorable postnatal outcome with significant dilation of the fetal main pulmonary artery near term.

Fetal echocardiography in third trimester is relatively rare reported and there is no data, what is the clinical meaning of the main pulmonary artery (MPA) dilatation in third trimester or before the delivery in fetuses with congenital heart defect. We analyzed the neonatal follow-up in cases of significantly dilated MPA diameter to better understand its clinical significance. Retrospectively 238 healthy singleton fetuses were selected as a reference group. Consecutive percentiles for MPA diameter according to the gestational age were calculated. In second step, we selected cases whose MPA diameter measured at the level of three vessel trachea view (3VT view) was pointedly above 95th centile in third trimester of pregnancy, according to the obtained data of our healthy population. There were 11 fetuses, with dilated MPA diameter (range 12-13.5 mm), who had last echocardiography performed at 37.6 weeks of gestation. There were 11 isolated heart defects: 7 cases with HLHS, 2 with d-TGA, and 2 cases with CoA. Mean neonatal weight was 3,345 g, with Apgar score 8-10. About 10 newborns out of 11 had cardiac surgery at the mean 12th day of the postnatal life and 8 of them died on the mean 23rd day. Autopsy was performed in 5 cases. In all of them histopathology reports described pulmonary hypertension in addition to cardiac structural abnormalities. Severe dilation of the fetal MPA before the delivery suggested prenatal abnormal lung development and was a poor prognostic factor. In these cases postnatal pulmonary hypertension should be suspected.

Prenatal Diagnosis of Fetal Trisomy 5 Mosaicism with Congenital Pulmonary Airway Malformation Type 3: A Case Report

Background: Trisomy mosaicism of chromosome 5 is uncommon with few cases described. Case report: A 41-year-old woman underwent ultrasound (US) at 16 weeks, which showed oligohydramnios and intrauterine growth restriction (IUGR). Amniocentesis discovered a karyotype of 47,XX,+5/46,XX. US at 19 weeks disclosed IUGR, enlargement of right side of heart, main pulmonary artery dilatation, and a suspected congenital pulmonary airway malformation (CPAM) in the inferior lobe of the left lung. Due to poor fetal prognosis, the parents opted for legal termination of pregnancy. At postmortem, a wide ventricular septal defect and CPAM type 3 were found. Cytogenetic analyses on fetal tissues detected mosaic trisomy 5 in skin, thymus, kidneys and CPAM. Placenta and fetal peripheral blood revealed normal female karyotype. Discussion/conclusion: These results suggest that if a fetus presents normal phenotypic features, mosaicism may be confined to extraembryonic structures, otherwise, in case of malformations, it may be carried by affected organs.

Transcatheter ASD closure in a patient with Dextroversion: A case report

Background: Cardiac dextroversion is location of the heart in the right chest with the left ventricle remaining in the normal position to the left but lying anterior to the right ventricle. To the best of our knowledge no specific technique has been described before for successful transcatheter secundum atrial septal defect closure in patients with dextroversion. Case presentation: We present a case of secundum atrial ASD in a patient with dextroversion, situssolitus, AV concordance and VA concordance. Patient was referred for transcatheter closure of ASD. Her transthoracic echocardiography showed a 7 mm secundum ASD, Upper normal RV size, small restrictive VSD 2 mm and a dilated main pulmonary artery. Transesophagealechocardiography showed an 11 mm defect with abnormal orientation of inter atrial septum due to cardiac dextroversion. Usual technique for positioning of ASD Amplatzer device (ASO 11) failed with prolapsing of the device into right atrium. Failed right upper pulmonary vein technique. With successful positioning of the device across the interatrial septum using left upper pulmonary vein technique. Conclusion: We recommend left upper pulmonary vein technique for safe and direct positioning of ASD device across interatrial septum in patients with dextroversion.

Clinical and diagnostic imaging findings in 37 rescued dogs with heartworm disease in Germany

Canine dirofilariasis is currently uncommon in Germany, but its prevalence appears to be increasing due to rehoming of dogs from endemic areas. As part of staging of the disease, this case series describes clinical, radiological and echocardiographic findings in 37 dogs tested positive for Dirofilaria immitis between October 2016 and January 2019. Clinical history revealed 12 (32 %) asymptomatic and 22 (59 %) symptomatic dogs, with most of the dogs showing exercise intolerance, coughing or breathlessness. Thoracic radiographs were performed in 34 dogs and showed parenchymal lesions in 14 (41 %) and bulging of the main pulmonary artery (MPA) in 8 (23,5 %). Enlarged peripheral pulmonary arteries were noted in 22 (65 %) dogs, mainly affecting caudal peripheral pulmonary arteries. In 12 (24 %) of dogs with echocardiographic findings, thoracic radiographs were unremarkable. Echocardiographic examination was performed to assess secondary pulmonary hypertension (PH), identification of worm burden and dilation of pulmonary arteries. RPAD was applied for grading of PH in 34 dogs. Normal values (RPAD > 35 %) were detected in 11 (32 %) dogs, mild PH (RPAD 28–35 %) in 12 (35 %), moderate PH (RPAD 23–27 %) in 5 (15 %), and severe PH (RPAD < 23 %) in 6 (18 %) dogs. Severe systolic PH was confirmed by pressure gradient of tricuspid regurgitation in two dogs. Pressure gradients of pulmonary insufficiency were suggestive of diastolic PH in 5 dogs and correlated with RPAD < 23 %. Right ventricular hypertrophy was observed in 6 dogs with severe PH, flattening of the interventricular septum in 5 dogs. Right ventricular systolic time intervals showed a short acceleration time (<80 ms) and AT/ET of <0.31 in all dogs with severe PH. MPA dilation, defined as the ratio of MPA to aorta (MPA/Ao) exceeding 1.0 was found in 16 (43 %) dogs. Right pulmonary artery (RPA) dilation, defined as the ratio of RPA to aorta (PRA/Ao) exceeding 0.5 was detected in 25 (68 %) dogs. Double, linear parallel objects (DL) suggestive for macrofilaria were found in 28 (76 %) dogs. Of these, 7 (19 %) had a high worm burden occupying RPA and MPA. Six months after the 3rd Melarsomine injection a follow up examination was performed in 21 dogs. Main findings included persistent dilation of RPA in 11 (52 %), as well as RPAD indicative of PH in 11 (52 %) dogs. This case series demonstrates that>70 % of dogs had at least one relevant finding suggestive of PH prior to starting adulticide treatment. Therefore, thoracic radiographs as well as a thorough echocardiographic examination are recommended in all dogs that test positive for D. immitis. Echocardiography should focus on presence and severity of PH, for which RPAD has been proven to be useful. A follow up examination after adulticide treatment should be performed, which provides comparative assessment of presence and severity of PH. Dogs with persistent moderate to severe PH may benefit from long-term monitoring and medical management.

Decreased size of the left anterior descending coronary artery is an independent predictor of deterioration in non-high-risk patients with acute pulmonary embolism.

To evaluate the efficacy of measuring left coronary artery size to predict deterioration in non-high-risk acute pulmonary embolism (PE) patients. This retrospective study enrolled non-high-risk acute PE patients from January 2011 to December 2019. Patient deterioration was defined as the occurrence of adverse events within 30days of hospital admission. Patients with adverse events were sex- and age-matched to patients without adverse events. Risk stratification was performed. Cross-sectional areas (CSAs) of the left main and left anterior descending (LAD) coronary artery inlets were measured. The main pulmonary artery (MPA) inlet and outlet and MPA LAD plane, which adjoined the LAD in the MPA, were reconstructed. CSAs, perimeters, and hydraulic diameters were measured to evaluate MPA size and deformation. Cardiac volume was also measured. Quantitative parameters were divided into tertiles. After adjustment by risk stratification, univariate and multivariate analyses were performed. Correlations between different parameters were analysed. Seventy-three patients with adverse events were matched to 73 patients without adverse events. The results of the univariate and multivariate analyses revealed that LAD inlet CSAs (middle and high) predicted adverse events (odds ratio: 0.28 and 0.07, 95% confidence interval: 0.10-0.77 and 0.02-0.22, p = 0.013 and < 0.0001). LAD inlet CSA was strongly and negatively correlated with MPA LAD hydraulic diameter and CSA (correlation coefficients: - 0.643 and - 0.604, p < 0.001). LAD inlet CSA measurement would facilitate adverse event prediction in non-high-risk acute PE patients on the basis of risk stratification. The dilated MPA may involve the decrease in LAD inlet CSA.

A Novel Predictive Method Incorporating Parameters of Main Pulmonary Artery Bifurcation for Short-Term Prognosis in Non-high-risk Acute Pulmonary Embolism Patients.

The aim of this study was to build a formula to predict short-term prognosis using main pulmonary artery (MPA) parameters reconstructed from computed tomographic pulmonary angiography in non-high-risk acute pulmonary embolism (PE) patients. After reconstructing the MPA and its centerline, the MPA, the right and left pulmonary artery inlet, and the MPA outlet plane were differentiated to measure the cross-sectional area (CSA), the maximal diameter and the hydraulic diameter. The MPA bifurcation area, volume and angle were measured. MPA dilation was defined as >29 mm at the transverse section plane. The patients were randomly divided into a training set and a validation set. A least absolute shrinkage and selection operator (LASSO) logistic regression algorithm was used to build a predictive formula. The performances of the predictive formula from LASSO were tested by the area under the receiver operating characteristic curve (AUC) and precision-recall (PR) curve with 10-fold cross-validation. The clinical utility was assessed by decision curve analysis (DCA). In total, 296 patients were enrolled and randomly divided (50:50) into a training set and a validation set. The LASSO predictive formula (lambda.1SE) was as follows: 0.92 × MPA bifurcation area + 0.50 × MPA outlet hydraulic diameter + 0.10 × MPA outlet CSA. The AUCs of the predictive formula were 0.860 (95% CI: 0.795–0.912) and 0.943 (95% CI: 0.892–0.975) in the training set and validation set, respectively. The LASSO predictive formula had a higher average area under the PR curve than MPA dilation (0.71 vs. 0.23 in the training set and 0.55 vs. 0.23 in the validation set) and added a net benefit in clinical utility by DCA. Integration of MPA outlet CSA, hydraulic diameter, and bifurcation area with the LASSO predictive formula as a novel weighting method facilitated the prediction of poor short-term prognosis within 30 days after hospital admission in non-high-risk acute PE patients.