MyMedR

Fulltext Unveiling the dual role of autophagy in vascular remodelling and its related diseases

Ren H, Dai R, Nik Nabil WN, Xi Z, Wang F, Xu H

Biomed Pharmacother, 2023 Dec;168:115643.

PMID: 37839111 DOI: 10.1016/j.biopha.2023.115643

Vascular remodelling is an adaptive response to physiological and pathological stimuli that leads to structural and functional changes in the vascular intima, media, and adventitia. Pathological vascular remodelling is a hallmark feature of numerous vascular diseases, including atherosclerosis, hypertension, abdominal aortic aneurysm, pulmonary hypertension and preeclampsia. Autophagy is critical in maintaining cellular homeostasis, and its dysregulation has been implicated in the pathogenesis of various diseases, including vascular diseases. However, despite emerging evidence, the role of autophagy and its dual effects on vascular remodelling has garnered limited attention. Autophagy can exert protective and detrimental effects on the vascular intima, media and adventitia, thereby substantially influencing the course of vascular remodelling and its related vascular diseases. Currently, there has not been a review that thoroughly describes the regulation of autophagy in vascular remodelling and its impact on related diseases. Therefore, this review aimed to bridge this gap by focusing on the regulatory roles of autophagy in diseases related to vascular remodelling. This review also summarizes recent advancements in therapeutic agents targeting autophagy to regulate vascular remodelling. Additionally, this review offers an overview of recent breakthroughs in therapeutic agents targeting autophagy to regulate vascular remodelling. A deeper understanding of how autophagy orchestrates vascular remodelling can drive the development of targeted therapies for vascular diseases.

Matched MeSH terms: Hypertension, Pulmonary*

Fulltext Primary pulmonary hypertension. World Health Organization

Fauzi ARM

Med J Malaysia, 2000 Dec;55(4):529-37; quiz 538.

PMID: 11221172

Primary pulmonary hypertension (PPH) is a rare disease. The annual incidence in the West is 1-2 cases per million population per year. A recent WHO symposium in 1998 has produced a consensus on classification, methods of screening, risk assessment and treatment. PPH is a diagnosis of exclusion after all other secondary causes of pulmonary hypertension are ruled out. Current treatment strategy involves acute vasodilator drug trial where positive responders are treated with high dose calcium channel blockers and anticoagulation. Those who do not show positive response may be commenced on intravenous prostacyclin. Surgical treatment is one option for patients with severe PPH or for symptomatic relief. Prognosis in general is very poor.

Matched MeSH terms: Hypertension, Pulmonary/classification; Hypertension, Pulmonary/diagnosis*; Hypertension, Pulmonary/epidemiology; Hypertension, Pulmonary/therapy*

Fulltext eComment. Paradigm shift in surgery for massive pulmonary embolism: if not now then when?

Sachithanandan A

Interact Cardiovasc Thorac Surg, 2013 Aug;17(2):246.

PMID: 23868961 DOI: 10.1093/icvts/ivt274

Matched MeSH terms: Hypertension, Pulmonary/surgery*

Pulmonary hypertension, cardiac disease and pregnancy

Tahir H

Int J Gynaecol Obstet, 1995 Nov;51(2):109-13.

PMID: 8635630

OBJECTIVE: To ascertain the significance of coexisting pulmonary hypertension in cardiac disease in pregnancy.

METHODS: Over a 3-year period a group of pregnant women with cardiac disease was followed until 6 weeks postpartum. Twenty women with pulmonary hypertension were compared with 20 controls without pulmonary hypertension with particular reference to maternal and fetal outcome. Analysis of data was carried out using Fisher's exact test and Student's t-test.

RESULTS: Except for Eisenmenger's syndrome, there were no differences in maternal morbidity and mortality between the two groups. There were more low birth weight babies but no significant differences in premature delivery rate, mode of delivery or perinatal mortality.

CONCLUSION: Except for Eisenmenger's syndrome, coexisting pulmonary hypertension complicating cardiac disease in pregnancy generally has a favorable outcome for both mother and fetus.

Matched MeSH terms: Hypertension, Pulmonary/complications*; Hypertension, Pulmonary/mortality

Fulltext Pulmonary valve echocardiogram in the evaluation of pulmonary arterial hypertension in the presence of intracardiac shunts

Fong CY, Singham KT

Med J Malaysia, 1986 Sep;41(3):198-204.

PMID: 3670135

The purpose of this study was to assess M-mode echocardiographic criteria for pulmonary artery hypertension in patients with intracardiac shunts, and to observe any signs not previously described.
Forty-seven consecutive cardiac cathetherised patients with routine M-mode echocardiogram done were reviewed. Twenty-six patients had atrial septal defect, nine had ventricular septal defect (VSD), four had patent ductus arteriosus (PDA), five had Eisenmenger syndrome and three had combined VSD and PDA.
Fifteen of 20 patients with pulmonary artery mean pressure (PAMP) of more than 50 mmHg demonstrated diminished a-dip (p < 0.001), reduced e-f slope (p < 0.01) and increased b-e slope to > 380 mm/sec (p < 0.01). The presence of systolic flutter or mid-systolic notch, or both was not observed in patients with normal PAMP, but was noted in all of the 20 patients with raised pressure(> 50 mmHg).
This study confirms the limitation of M-mode echocardiographic features in the evaluation of pulmonary artery hypertension in patients with intracardiac shunt, though clinically useful.

Matched MeSH terms: Hypertension, Pulmonary/diagnosis; Hypertension, Pulmonary/physiopathology*

Hepatopulmonary Syndrome and Portopulmonary Hypertension in Children: Recent Advances in Diagnosis and Management

Lee WS, Wong SY, Ivy DD, Sokol RJ

J Pediatr, 2018 05;196:14-21.e1.

PMID: 29514741 DOI: 10.1016/j.jpeds.2017.12.068

Matched MeSH terms: Hypertension, Pulmonary/diagnosis*; Hypertension, Pulmonary/therapy*

Atrial septal defect coexistent with Sjögren's syndrome

Ahmad F, Sadiq MA, Chee KH, Mahmood Zuhdi AS, Wan Ahmad WA

J Coll Physicians Surg Pak, 2014 Jun;24(6):441-3.

PMID: 24953923 DOI: 06.2014/JCPSP.441443

Pulmonary hypertension is frequently associated with atrial septal defect and various connective tissue disorders. This case describes a 74-year-old woman who presented with symptoms of heart failure and concomitant involvement of salivary glands and keratoconjunctivitis. An echocardiogram demonstrated ostium secundum atrial septal defect with left to right shunt and severe pulmonary hypertension. Laboratory investigations confirmed the diagnosis of Sjögren's syndrome (SS) with positive anti-nuclear factor and centromere SS-A/Ro pattern. Anti-Ro (SS-A) was found positive. Atrial septal defect was closed through transcatheter route with significant improvement in clinical outcome. This case report suggests a possible association of atrial septal defect with primary Sjögren's syndrome in an adult patient.

Matched MeSH terms: Hypertension, Pulmonary/complications*; Hypertension, Pulmonary/therapy

Sildenafil as treatment for Human Immunodeficiency Virus-related pulmonary hypertension in a child

Wong AR, Rasool AH, Abidin NZ, Noor AR, Quah BS

J Paediatr Child Health, 2006 Mar;42(3):147-8.

PMID: 16509918

Human Immunodeficiency Virus (HIV)-related pulmonary hypertension is a relatively rare disease that can affect HIV sufferers. This is almost always associated with a poor outcome and death. An 18 month-old girl, probably the youngest on record, was diagnosed to have pulmonary hypertension (PHT) and retrospectively found to have HIV infection. Sildenafil was used to control her PHT and she remains alive even after 2 years.

Matched MeSH terms: Hypertension, Pulmonary/drug therapy*; Hypertension, Pulmonary/etiology

Fulltext The frequency of pulmonary hypertension in newborn with intrauterine growth restriction

Abbas G, Shah S, Hanif M, Shah A, Rehman AU, Tahir S, et al.

Sci Rep, 2020 05 15;10(1):8064.

PMID: 32415157 DOI: 10.1038/s41598-020-65065-2

Intrauterine growth restriction (IUGR) is a clinical definition applied to neonates born with clinical features of malnutrition and in-utero growth retardation irrespective of their birth weight percentile. This study was aimed to determine the frequency of pulmonary hypertension (PH) in neonates with IUGR. In this descriptive cross-sectional study, we followed 96 neonates with IUGR (≤28 days) and 38 neonates without IUGR born in the department of the neonatal intensive care unit children hospital complex Multan, Pakistan. We analyzed certain factors such as gender, gestational age (GA) (weeks), birth weight (BW in kg), weight percentile (WP) for GA, meconium aspiration syndrome (MAS), birth asphyxia (BA) and respiratory distress syndrome (RDS) for pulmonary hypertension (PH) in IUGR and non-IUGR group. GA was measured by the Ballard scoring system. Echocardiography was performed for all patients by the pediatric cardiologist to measure pulmonary arterial (PA) pressure using Bernoulli's equation. Out of total 96 IUGR neonates, 33.3% (n = 32) suffered from PH, of which 65.3% (n = 18) were male and 43.7% (n = 14) were female. The percentages of IUGR neonates with BA, MAS and RDS were 34.4%, 18.8% and 22.9% respectively. The data were analyzed using the SPSS-16 software to test the statistical significance of the results. A p-value less than 0.05 was considered significant. When the chi-square test was applied, it depicted that MAS was significantly associated with PH in IUGR neonates (p = 0.0001) compared to non-IUGR neonates. Our findings suggested an increased chance of PH in IUGR neonates and MAS may be a strong factor.

Matched MeSH terms: Hypertension, Pulmonary/epidemiology*; Hypertension, Pulmonary/pathology

Fulltext Unresolved tachypnoea: a presentation of congenital cor-triatriatum

Fahisham Taib, Nur Atiqah Abdul Rahman

Malaysian Journal of Paediatrics and Child Health, 2019;25(2):23-27.

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: Hypertension, Pulmonary

Pulmonary hypertension complicating systemic sclerosis: A successful pregnancy outcome

Chew KT, Ahmad MF, Yong SL, Mohamed Ismail NA

J Obstet Gynaecol, 2017 Jul;37(5):677-678.

PMID: 28350528 DOI: 10.1080/01443615.2017.1284774

Matched MeSH terms: Hypertension, Pulmonary

Fulltext Early outcome of congenital diaphragmatic hernia in a Malaysian tertiary centre

Rohana J, Boo NY, Thambidorai CR

Singapore Med J, 2008 Feb;49(2):142-4.

PMID: 18301842

This prospective observational study was conducted to determine the outcome of newborns with congenital diaphragmatic hernia (CDH). They were managed with a protocol of gentle ventilation to avoid barotraumas, and inhaled nitric oxide (iNO) or intravenous magnesium sulphate for treatment of persistent pulmonary hypertension of newborns (PPHN).

Matched MeSH terms: Hypertension, Pulmonary/complications; Hypertension, Pulmonary/drug therapy*; Hypertension, Pulmonary/mortality

Fulltext Pulmonary haemosiderosis with juvenile idiopathic arthritis in a Malaysian child

Wong AR, Noor AS, Rasool AH, Quah BS, Roberton D

Med J Malaysia, 2007 Oct;62(4):352-4.

PMID: 18551948 MyJurnal

A rare case of childhood pulmonary haemosiderosis with juvenile idiopathic arthritis is discussed, with particular reference to treatment with hydroxychloroquine and sildenafil for pulmonary hypertension which occurs secondary to this disease.

Matched MeSH terms: Hypertension, Pulmonary/drug therapy; Hypertension, Pulmonary/etiology*

Fulltext Stenting of vertical vein in an infant with obstructed supracardiac total anomalous pulmonary venous drainage

Lim WK, Leong MC, Samion H

Ann Pediatr Cardiol, 2016 5 24;9(2):183-5.

PMID: 27212859 DOI: 10.4103/0974-2069.173549

A 1.7 kg infant with obstructed supracardiac total anomalous pulmonary venous drainage (TAPVD) presented with severe pulmonary hypertension secondary to vertical vein obstruction. The child, in addition, had a large omphalocele that was being managed conservatively. The combination of low weight, unoperated omphalocele, and severe pulmonary hypertension made corrective cardiac surgery very high-risk. Therefore, transcatheter stenting of the stenotic vertical vein, as a bridge to corrective surgery was carried out. The procedure was carried out through the right internal jugular vein (RIJ). The stenotic segment of the vertical vein was stented using a coronary stent. After procedure, the child was discharged well to the referred hospital for weight gain and spontaneous epithelialization of the omphalocele. Stenting of the vertical vein through the internal jugular vein can be considered in very small neonates as a bridge to repair obstructed supracardiac total anomalous venous drainage.

Matched MeSH terms: Hypertension, Pulmonary

Fulltext Factors Affecting the Quality of Sleep in Children

Fadzil A

Children (Basel), 2021 Feb 09;8(2).

PMID: 33572155 DOI: 10.3390/children8020122

Sleep quality is one of the domains of sleep. Having adequate quality sleep is defined as one's "feeling fresh" after waking-up. Inadequate sleep quality results in sleep insufficiency producing a variety of symptoms and signs. The central nervous system is affected the most in children, although other system too may be involved. Several factors affect sleep quality in children including genetics, sleep habits, medical problems, parents/caregiver factors, screen time and the child's environment. These factors are inter-related and dynamic. The outcome of sleep insufficiency is many involving neurocognitive and neurobehavior, mood and emotional issues and specific conditions, like pulmonary hypertension, cor pulmonale and obesity. Management should start with proper history taking to identify the multifaceted nature of the condition. Treatment is planned cognizant of the age of the patient and the associated etiological factors, and should involve both the children and their parents.

Matched MeSH terms: Hypertension, Pulmonary

Prognostic factors associated with survival of patients with ventricular septal defect

Suhaimi Hussain, Wong A. Rahim, Ibrahim W. Pauzi W., Naing, N.N.

Malaysian Journal of Paediatrics and Child Health, 2007;15(1):16-23.

MyJurnal

Introduction: There are many factors that determine the survival of patients with VSD. Among these include size of VSD, position, pulmonary hypertension, bacterial sepsis, valvular involvement, associated anomalies with VSD, associated syndromes and age at first diagnosis. There has been no published local data as far as we know and this information will be useful especially for consultation with parents. Even though VSD in general has a good prognosis, whenever they have added risks for example pulmonary hypertension then they are at risk of further morbidity and mortality. Objective: To determine the factors that are associated with survival of patients with VSD. Design: Retrospective cohort. Materials and methods: All cases of isolated VSDs admitted to HUSM from 1996 to 2003 were reviewed. Results: Univariate Cox regression of survival time of patients with VSDs revealed that 4 factors had prognostic significance namely bacterial sepsis (HR= 287.7, 95% CI 51.1, 1618.5, P < 0.001), Down syndrome (HR = 14.89, 95% CI 3.00, 73.92, P = 0.001), pulmonary hypertension (HR=14.58, 95% CI 1.69, 125.7, P=0.015) and large VSDs (HR=8.23, 95% CI 1.5, 45, P=0.015). Bacterial sepsis was the only significant prognostic factors for the survival of patients with VSDs using the multivariate Cox proportional hazard model. Conclusion: Bacterial sepsis, pulmonary hypertension, large VSD and Down syndrome were the significant prognostic factors from Univariate Cox analysis, however bacterial sepsis was the only significant prognostic factor from Multivariate Cox analysis.

Matched MeSH terms: Hypertension, Pulmonary

Progressive pulmonary vein stenosis in Down syndrome infant: a rare cause of pulmonary hypertension

Norazah Z, Joyce Darshinee DS

Med J Malaysia, 2018 04;73(2):119-120.

PMID: 29703879 MyJurnal

Pulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a Down Syndrome infant with congenital heart disease and transient myeloproliferative leukaemia. This case-report aims to improve awareness among physicians and sonographers of this disease and the importance of pulmonary vein assessment in congenital heart disease with unexplained pulmonary hypertension.

Matched MeSH terms: Hypertension, Pulmonary

Fulltext Severe heartworm disease with pulmonary hypertension in a local dog

Leong, Z.P., Watanabe, M., Lim, M.L.

Jurnal Veterinar Malaysia, 2015;27(1):27-30.

MyJurnal

A 4-year-old, male local dog was referred to University Veterinary Hospital-Universiti Putra Malaysia (UVH-UPM) due to
dyspnoea and ascites. Full diagnostic investigations inclusive of blood haematology, serum biochemistry, blood smear examinations
for haemopathogens, heartworm antigen test, thoracic and abdominal radiography and echocardiography were conducted. A
diagnosis of severe heartworm disease was made from the positive heartworm antigen test, the presence of heartworms on
echocardiography and the accompanying advanced clinical findings. This was the first case of right-sided heart failure due to severe
heartworm disease with concurrent pulmonary hypertension diagnosed in UVH-UPM from which the moribund dog was
successfully stabilised during hospitalisation.

Matched MeSH terms: Hypertension, Pulmonary

Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital

Teh CL, Kuan YC, Wong JS

Rheumatol Int, 2009 Aug;29(10):1243-5.

PMID: 19373465 DOI: 10.1007/s00296-009-0938-z

We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.

Matched MeSH terms: Hypertension, Pulmonary/diagnosis*

Bariatric surgery in patients with pulmonary hypertension

Hanipah ZN, Mulcahy MJ, Sharma G, Punchai S, Steckner K, Dweik R, et al.

Surg Obes Relat Dis, 2018 Oct;14(10):1581-1586.

PMID: 30449514 DOI: 10.1016/j.soard.2018.07.015

BACKGROUND: Data regarding the outcomes of bariatric surgery in patients with pulmonary hypertension (PH) is limited. The aim of this study was to review our experience on bariatric surgery in patients with PH.

SETTING: An academic medical center.

METHODS: Patients with PH who underwent either a primary or revisional bariatric surgery between 2005 and 2015 and had a preoperative right ventricle systolic pressure (RVSP) ≥35 mm Hg were included.

RESULTS: Sixty-one patients met the inclusion criteria. Fifty (82%) were female with the median age of 58 years (interquartile range [IQR] 49-63). The median body mass index was 49 kg/m2 (IQR 43-54). Procedures performed included the following: Roux-en-Y gastric bypass (n = 33, 54%), sleeve gastrectomy (n = 24, 39%), adjustable gastric banding (n = 3, 5%), and banded gastric plication (n = 1, 2%). Four patients (7%) underwent revisional bariatric procedures. Median operative time and length of stay was 130 minutes (IQR 110-186) and 3 days (IQR 2-5), respectively. The 30-day complication rate was 16% (n = 10) with pulmonary complications noted in 4 patients. There was no 30-day mortality. One-year follow-up was available in 93% patients (n = 57). At 1 year, median body mass index and excess weight loss were 36 kg/m2 (IQR 33-41) and 51% (IQR 33-68), respectively. There was significant improvement in the RVSP after bariatric surgery at a median follow-up of 22 months (IQR 10-41). The median RVSP decreased from 44 (IQR 38-53) to 40 mm Hg (IQR 28-54) (P = .03).

CONCLUSION: Bariatric surgery can be performed without prohibitive complication rates in patients with PH. In our experience, bariatric patients with PH achieved significant weight loss and improvement in RVSP.

Matched MeSH terms: Hypertension, Pulmonary/complications*

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