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  1. Abdul Latiff H, Gopal AR, Hidayat ZF, Haranal M, Borhanuddin BK, Alwi M, et al.
    Cardiol Young, 2023 Nov;33(11):2243-2251.
    PMID: 36651340 DOI: 10.1017/S1047951122004218
    BACKGROUND: The objective was to study the ductus arteriosus morphology in duct-dependent pulmonary circulation and its pattern in different ventricle morphology using CT angiography.

    METHOD: From January 2013 to December 2015, patients aged 6 months and below with duct-dependent pulmonary circulation underwent CT angiography to delineate the ductus arteriosus origin, tortuosity, site of insertion, and pulmonary artery anatomy. The ductus arteriosus were classified into type I, IIa, IIb, and III based on its site of origin, either from descending aorta, distal arch, proximal arch, or subclavian artery, respectively.

    RESULTS: A total of 114 patients and 116 ductus arteriosus (two had bilateral ductus arteriosus) were analysed. Type I, IIa, IIb, and III ductus arteriosus were seen in 13 (11.2 %), 71 (61.2%), 21 (18.1%), and 11 (9.5%), respectively. Tortuous ductus arteriosus was found in 38 (32.7%), which was commonly seen in single ventricular lesions. Ipsilateral and bilateral branch pulmonary artery stenosis was seen in 68 (59.6%) and 6 (5.3%) patients, respectively. The majority of patients with pulmonary atresia intact ventricular septum had type I (54.4%) and non-tortuous ductus arteriosus, while those with single and biventricular lesions had type II ductus arteriosus (84.9% and 89.7%, respectively). Type III ductus arteriosus was more common in biventricular lesions (77.8%).

    CONCLUSIONS: Ductus arteriosus in duct-dependent pulmonary circulation has a diverse morphology with a distinct origin and tortuosity pattern in different types of ventricular morphology. CT may serve as an important tool in case selection and pre-procedural planning for ductal stenting.

    Matched MeSH terms: Pulmonary Circulation
  2. Fahisham Taib, Nur Atiqah Abdul Rahman
    MyJurnal
    Cor-triatriatum is a rare cardiac anomaly. In literature, majority case reports on the condition focused on its late presentation in adulthood. It can be easily corrected by surgical intervention to avoid pulmonary congestion and subsequent pulmonary hypertension. We report a rare case of cor-triatriatum with severe pulmonary hypertension in a 7-week-old baby who presented with persistent tachypnoea.
    Matched MeSH terms: Pulmonary Circulation
  3. Alwi M, Choo KK, Latiff HA, Kandavello G, Samion H, Mulyadi MD
    J Am Coll Cardiol, 2004 Jul 21;44(2):438-45.
    PMID: 15261945 DOI: 10.1016/j.jacc.2004.03.066
    Objectives: This study was designed to assess the safety, efficacy, medium-term outcome, and complications of patent ductus arteriosus (PDA) stenting in duct-dependent pulmonary circulation.
    Background: Patent ductus arteriosus stenting has been proposed as an alternative to surgical shunt on account of postoperative morbidity and complications of surgical shunting.
    Methods: Between April 2000 and February 2003, 69 patients with duct-dependent pulmonary circulation underwent cardiac catheterization with the intent of PDA stenting as first palliative procedure. Patients with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum post-radiofrequency valvotomy who had PDA stenting were excluded. Thirteen more patients were excluded because of branch pulmonary artery (PA) stenosis. The follow-up was by clinical examination, echocardiography, and repeat cardiac catheterization at six to nine months following the procedure.
    Results: Patent ductus arteriosus stenting was successful in 51 patients (91.1%) and failed in 5 patients (8.9%). The mean narrowest PDA diameter was 1.9 +/- 0.6 mm. The mean procedure and fluoroscopy time were 95.7 min and 29.4 min, respectively. In one patient the stent dislodged and migrated to the left femoral artery and another patient developed transient intravascular hemolysis. There was no procedure-related mortality. Three patients (5.9%) died one day to two months after the procedure. At follow-up (3.2 months to 2.4 years), 8 patients developed significant stent stenosis requiring reintervention. Seven patients developed worsening of preexisting branch PA stenosis. The freedom from reintervention was 89% and 55% at 6 months and 1 year, respectively.
    Conclusions: Patent ductus arteriosus stenting is an attractive alternative to surgical shunt in a majority of patients with duct-dependent circulation. An absolute contraindication to this technique is the presence of branch pulmonary stenosis.
    Matched MeSH terms: Pulmonary Circulation*
  4. Tan TL, Illa NE, Ting SY, Hwong PL, Azmel A, Shunmugarajoo A, et al.
    Med J Malaysia, 2023 Mar;78(2):155-162.
    PMID: 36988524
    INTRODUCTION: The co-existence of coronavirus disease 2019 (COVID-19) and pulmonary thromboembolic (PTE) disease poses a great clinical challenge. To date, few researches have addressed this important clinical issue among the South-East Asian populations. The objectives of this study were as follow: (1) to describe the clinical characteristics and computed tomographical (CT) features of patients with PTE disease associated with COVID-19 infection and (2) to compare these parameters with those COVID-19 patients without PTE disease.

    MATERIALS AND METHODS: This cross-sectional study with retrospective record review was conducted in Hospital Tengku Ampuan Rahimah, Selangor, Malaysia. We included all hospitalised patients with confirmed COVID-19 infection who had undergone CT pulmonary angiogram (CTPA) examinations for suspected PTE disease between April 2021 and May 2021. Clinical data and laboratory data were extracted by trained data collectors, whilst CT images retrieved were analysed by a senior radiologist. Data analysis was performed using Statistical Package for the Social Sciences (SPSS) version 20.

    RESULTS: We studied 184 COVID-19 patients who were suspected to have PTE disease. CTPA examinations revealed a total of 150 patients (81.5%) suffered from concomitant PTE disease. Among the PTE cohort, the commonest comorbidities were diabetes mellitus (n=78, 52.0%), hypertension (n=66, 44.0%) and dyslipidaemia (n=25, 16.7%). They were generally more ill than the non-PTE cohort as they reported a significantly higher COVID-19 disease category during CTPA examination with p=0.042. Expectedly, their length of both intensive care unit stays (median number of days 8 vs. 3; p=0.021) and hospital stays (median number of days 14.5 vs. 12; p=0.006) were significantly longer. Intriguingly, almost all the subjects had received either therapeutic anticoagulation or thromboprophylactic therapy prior to CTPA examination (n=173, 94.0%). Besides, laboratory data analysis identified a significantly higher peak C-reactive protein (median 124.1 vs. 82.1; p=0.027) and ferritin levels (median 1469 vs. 1229; p=0.024) among them. Evaluation of CT features showed that COVID-19 pneumonia pattern (p<0.001) and pulmonary angiopathy (p<0.001) were significantly more profound among the PTE cohort. To note, the most proximal pulmonary thrombosis was located in the segmental (n=3, 2.0%) and subsegmental pulmonary arteries (n=147, 98.0%). Also, the thrombosis predominantly occurred in bilateral lungs with multilobar involvement (n=95, 63.3%).

    CONCLUSION: Overall, PTE disease remains prevalent among COVID-19 patients despite timely administration of thromboprophylactic therapy. The presence of hyperinflammatory activities, unique thrombotic locations as well as concurrent pulmonary parenchyma and vasculature aberrations in our PTE cohort implicate immunothrombosis as the principal mechanism of this novel phenomenon. We strongly recommend future researchers to elucidate this important clinical disease among our post- COVID vaccination populations.

    Matched MeSH terms: Pulmonary Circulation
  5. Alwi M
    Future Cardiol, 2012 Mar;8(2):237-50.
    PMID: 22413983 DOI: 10.2217/fca.12.4
    Maintaining ductal patency in duct-dependent congenital heart lesions by implantation of coronary stents is an alternative to systemic pulmonary shunt in selected cases and lesions with suitable anatomy. This article focuses on the procedure as the initial palliation in duct-dependent pulmonary circulation, its associated pitfalls and complications. A good understanding of the diverse duct morphology is paramount prior to stenting of the ductus. Long tortuous duct, insufficiently constricted ductus at the pulmonary end and ductus with associated branch pulmonary artery stenosis at the site of insertion are not suitable for stenting. Durability of palliation is generally inferior to a surgical shunt and this may dictate earlier definitive surgical repair. Acceleration of branch pulmonary artery stenosis in certain ductal morphology limits its general applicability. Bioabsorbable and biodegradable stents may offer some solution to this problem.
    Matched MeSH terms: Pulmonary Circulation*
  6. Lim E, Chan GS, Dokos S, Ng SC, Latif LA, Vandenberghe S, et al.
    PLoS One, 2013;8(10):e77357.
    PMID: 24204817 DOI: 10.1371/journal.pone.0077357
    A lumped parameter model of the cardiovascular system has been developed and optimized using experimental data obtained from 13 healthy subjects during graded head-up tilt (HUT) from the supine position to [Formula: see text]. The model includes descriptions of the left and right heart, direct ventricular interaction through the septum and pericardium, the systemic and pulmonary circulations, nonlinear pressure volume relationship of the lower body compartment, arterial and cardiopulmonary baroreceptors, as well as autoregulatory mechanisms. A number of important features, including the separate effects of arterial and cardiopulmonary baroreflexes, and autoregulation in the lower body, as well as diastolic ventricular interaction through the pericardium have been included and tested for their significance. Furthermore, the individual effect of parameter associated with heart failure, including LV and RV contractility, baseline systemic vascular resistance, pulmonary vascular resistance, total blood volume, LV diastolic stiffness and reflex gain on HUT response have also been investigated. Our fitted model compares favorably with our experimental measurements and published literature at a range of tilt angles, in terms of both global and regional hemodynamic variables. Compared to the normal condition, a simulated congestive heart failure condition produced a blunted response to HUT with regards to the percentage changes in cardiac output, stroke volume, end diastolic volume and effector response (i.e., heart contractility, venous unstressed volume, systemic vascular resistance and heart rate) with progressive tilting.
    Matched MeSH terms: Pulmonary Circulation/physiology
  7. Haranal M, Mood MC, Leong MC, Febrianti Z, Abdul Latiff H, Samion H, et al.
    Interact Cardiovasc Thorac Surg, 2020 08 01;31(2):221-227.
    PMID: 32437520 DOI: 10.1093/icvts/ivaa069
    OBJECTIVES: This study aims to review our institutional experience of ductal stenting (DS) on the growth of pulmonary arteries (PAs) and surgical outcomes of PA reconstruction in this subset of patients.

    METHODS: This is a retrospective study done in neonates and infants up to 3 months of age with duct-dependent pulmonary circulation who underwent DS from January 2014 to December 2015. Post-stenting PA growth, surgical outcomes of PA reconstruction, post-surgical re-interventions, morbidity and mortality were analysed.

    RESULTS: During the study period, 46 patients underwent successful DS, of whom 38 underwent presurgery catheterization and definite surgery. There was significant growth of PAs in these patients. Biventricular repair was done in 31 patients while 7 had univentricular palliation. Left PA augmentation was required in 13 patients, and 10 required central PA augmentation during surgery. The mean follow-up period post-surgery was 4.5 ± 1.5 years. No significant postoperative complications were seen. No early or follow-up post-surgery mortality was seen. Four patients required re-interventions in the form of left PA stenting based on the echocardiography or computed tomography evidence of significant stenosis.

    CONCLUSIONS: DS provides good short-term palliation and the growth of PAs. However, a significant number of stented patients require reparative procedure on PAs at the time of surgical intervention. Acquired changes in the PAs following DS may be the reason for reintervention following PA reconstruction.

    Matched MeSH terms: Pulmonary Circulation/physiology*
  8. Chan PWK, DeBruyne JA, Goh AYT, Muridan R
    Med J Malaysia, 1999 Dec;54(4):520-2.
    PMID: 11072474
    Swyer-James-MacLeod syndrome is a rare complication of respiratory tract infection occurring in early childhood. We report two children with chronic cough and recurrent wheezing who fulfilled the diagnostic criteria for this disorder: 1) Unilateral loss of lung volume with hyperlucency on chest x-ray. 2) Unilateral reduction in vascularity on CT scan of the chest. 3) Unilateral loss of perfusion on Technetium 99c lung scan.
    Matched MeSH terms: Pulmonary Circulation
  9. Mat Bah MN, Sapian MH, Alias EY
    Ann Pediatr Cardiol, 2020 09 17;13(4):320-326.
    PMID: 33311920 DOI: 10.4103/apc.APC_35_20
    Aims: There are limited data regarding critical congenital heart disease (CCHD) from middle-income countries (MIC). This study aims to determine the birth prevalence, rate of late diagnosis, and influence of timing of diagnosis on the outcome of CCHD.

    Setting and Design: Retrospective observational cohort study in the State of Johor, Malaysia.

    Subjects and Methods: All infants born between January 2006 and December 2015 with a diagnosis of CCHD, defined as infants with duct-dependent lesions or cyanotic heart disease who may die without early intervention. The late diagnosis was defined as a diagnosis of CCHD after 3 days of age.

    Results: Congenital heart disease was diagnosed in 3557 of 531,904 live-born infants and were critical in 668 (18.7%). Of 668, 347 (52%) had duct-dependent pulmonary circulation. The birth prevalence of CCHD was 1.26 (95% confidence interval: 1.16-1.35) per 1000 live births, with no significant increase over time. The median age of diagnosis was 4 days (Q1 1, Q3 26), with 61 (9.1%) detected prenatally, and 342 (51.2%) detected late. The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction of late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CCHD.

    Conclusions: Due to limited resources in the MIC, the late diagnosis of CCHD is high but does not affect the outcome. Nevertheless, the timing of diagnosis has improved over time.

    Matched MeSH terms: Pulmonary Circulation
  10. Nair AK, Haranal M, Elkhatim IM, Dillon J, Hew CC, Sivalingam S
    Ann Pediatr Cardiol, 2020 06 23;13(3):212-219.
    PMID: 32863656 DOI: 10.4103/apc.APC_111_19
    Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid-term outcomes of surgical correction of patients with APVS.

    Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients.

    Results: The overall 10-year survival was 82.1%. There was 81.1% overall freedom from re-intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re-intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10-year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications.

    Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems.

    Matched MeSH terms: Pulmonary Circulation
  11. Talib N, Sayuti KA, Abdullah MS, Mohd Zain MR
    BMJ Case Rep, 2018 Mar 05;2018.
    PMID: 29507009 DOI: 10.1136/bcr-2017-221074
    Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage. Cardiac CT confirmed supracardiac TAPVR, whereby all the pulmonary veins drain into the anomalous vein and finally to the superior vena cava. She remained asymptomatic and underwent a successful surgical repair.
    Matched MeSH terms: Pulmonary Circulation
  12. Riahi M, Ang HL, Jones M, Prachasilchai P, Baruteau AE, Promphan W, et al.
    Circ Cardiovasc Interv, 2018 04;11(4):e005923.
    PMID: 29618579 DOI: 10.1161/CIRCINTERVENTIONS.117.005923
    Matched MeSH terms: Pulmonary Circulation*
  13. Yang CY, Hoong MF, Li CS, Li WF, You SH, Lee YC, et al.
    Taiwan J Obstet Gynecol, 2021 May;60(3):517-522.
    PMID: 33966739 DOI: 10.1016/j.tjog.2021.03.023
    OBJECTIVE: To evaluate the association between intrauterine growth restriction (IUGR) and the incidence of fetuses with patent ductus arteriosus (PDA) and Hemodynamically significant PDA (Hs-PDA) in dichorionic twins (DC) with selective IUGR.

    MATERIALS AND METHODS: This is an observational cohort study and retrospective case assessment, involved twins born at Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan between 2013 and 2018. DC twins with selective IUGR (sIUGR) were defined as the presence of a birth weight discordance of >25% and a smaller twin with a birth weight below the tenth percentile. PDA was diagnosed using echocardiography between postnatal day 3 and 7. Hs-PDA was defined as PDA plus increased pulmonary circulation, poor systemic perfusion, cardiomegaly, pulmonary edema, or hypotension requiring pharmacotherapeutic intervention.

    RESULT: A total of 1187 twins were delivered during the study period, and 53 DC twins with selective IUGR were included in this study. DC twins with PDA have higher rate of preterm birth, lower gestational age of delivery, and lower mean birth weight of both twins compared with DC twins without PDA. In a comparison of the sIUGR twin with the appropriate for gestational age co-twin, both the incidences of PDA (28.30% vs. 7.55%, respectively; P = 0.003) and Hs-PDA (24.53% vs. 5.66%, respectively; P = 0.002) were higher in sIUGR fetuses than in the appropriate for gestational age co-twins. Small gestational age of delivery was the only variable to predict PDA and Hs-PDA [p = 0.002, Odds ratio = 0.57 (0.39-0.82), p = 0.009, Odds ratio = 0.71 (0.55-0.92), respectively].

    CONCLUSION: An analysis of dichorionic twins with sIUGR indicated that IUGR increased the risk of PDA and hemodynamically significant PDA.

    Matched MeSH terms: Pulmonary Circulation
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