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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*
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 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 Technique of anaesthesia in pulmonary hypertension and thrombophilia in early pregnancy

Chuah KH, Mansor M, Rajen G, Wang CY, Chan YK

Med J Malaysia, 2006 Mar;61(1):114-6.
PMID: 16708749 MyJurnal

Pulmonary hypertension in pregnancy is a rare condition but is associated with a high mortality. We report the case of a 29 year old female in early pregnancy with Protein C and S deficiency with recurrent deep venous thrombosis and pulmonary embolism and subsequent secondary pulmonary hypertension. The patient was counselled and consented for termination of pregnancy with tubal sterilization. She was administered continuous spinal anaesthesia with invasive monitoring. The successful anaesthetic management of this condition is described.

Matched MeSH terms: Hypertension, Pulmonary/etiology*
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 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*
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
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
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*
Fulltext Industry and cosmetic uses of talc with their implication on health

Sinniah, Davendralingam

International e-Journal of Science, Medicine & Education, 2011;5(1):10-16.
MyJurnal

Talc’s softness, whiteness, lamellarity, inertness and affinity for organic chemicals make it valuable for industrial and domestic applications. The largest consumers are the paper and ceramic industry; only 5% is used as cosmetics. It is also used for preserving animal feed, and a carrier for drugs, insecticides, pesticides and chemicals. Talc was introduced as baby powder in 1894 and advertised aggressively worldwide. Widespread and indiscriminate use soon raised concerns about its implications for health. The IARC found that talc containing asbestiform fibres is carcinogenic to humans, but inadequate evidence to implicate talc not-containing asbestiform fibres. Pulmonary manifestations of talc inhalation include talcosis, talcosilicosis, and talcoasbestosis. Drug-users administering talc-adulterated oral medications intravenously develop pulmonary granulomas, fibrosis and irreversible pulmonary hypertension. Worldwide reports reveal talc inhalation is fatal to infants; it coats and dries mucus membranes, causes hemorrhage, edema, desquamation of bronchial epithelium, and clogs and compromises mucociliary clearance; larger quantities completely obstruct airways. Progressive diffuse pulmonary fibrosis is a recognized sequel to massive aspiration of baby powder. IARC has classified perineal use of talcum powder as a possible ovarian carcinogen, while a recent study has found that perineal talcum powder increases the risk of endometrial cancer among postmenopausal women. There is a need to raise public awareness of the serious risks associated with the use of talcum powder and for legislation to protect the health of the uninformed who represent the poorer segment of the community, and infants and young children. The dangers associated with cosmetic use of talc outweigh any possible benefits.

Matched MeSH terms: Hypertension, Pulmonary
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*
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
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
Fulltext Pregnancy with hypoplastic left lung complicated by pneumothorax and pulmonary embolism

Zainudin LD, Abdul Hafidz MI, Zakaria AF, Mohd Zim MA, Ismail AI, Abdul Rani MF

Respirol Case Rep, 2016 Mar;4(1):19-21.
PMID: 26839696 DOI: 10.1002/rcr2.143

We report a case of a 34-year-old lady with past history of asthma and pulmonary tuberculosis, who presented 5 weeks pregnant with acute dyspnea. Her chest X-ray showed left-sided complete lung collapse and concomitant right-sided pneumothorax. The pneumothorax was initially managed conservatively with a chest tube but due to its persistence despite suction, was subsequently changed to a Pneumostat(TM), with which she was later discharged. She had a normal echocardiography (ejection fraction [EF] 67%) at 5 weeks of gestation but developed pulmonary hypertension (EF 55%, pulmonary arterial pressure 40.7 mmHg) as the pregnancy progressed. She delivered a healthy baby at 35 weeks via elective lower section caesarean section with spinal anesthesia. We followed her up postnatally and noted the presence of left-sided pulmonary embolism, hypoplastic left lung, and left pulmonary artery. The management of this complex case involved a multidisciplinary effort between general medical, respiratory, obstetric, and cardiothoracic teams.

Matched MeSH terms: Hypertension, Pulmonary
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
Pulmonary hypertension and echocardiogram parameters in obstructive sleep apnea

Wong HT, Chee KH, Chong AW

Eur Arch Otorhinolaryngol, 2017 Jun;274(6):2601-2606.
PMID: 28243782 DOI: 10.1007/s00405-017-4491-1

Obstructive sleep apnea (OSA) is a growing health hazard in the United States and worldwide. OSA is now recognized as a disorder with systemic manifestations and its association with obesity and adverse cardiovascular consequences. There is increasing evidence that OSA may be associated with systemic hypertension and an increased incidence of stroke, heart failure, myocardial infarction, and arrhythmias. Less information is available about the association between OSA and pulmonary hypertension (PH). We therefore conduct this study to look at the prevalence of the pulmonary hypertension in obstructive sleep apnea patient and to identify risk factors leading to pulmonary hypertension among OSA patient. We studied and analyzed all OSA patient confirmed by polysomnograph in the year 2015. Twenty-five patients with OSA were included in this study with prevalence of pulmonary hypertension of 16%. Univariate analysis of various factors revealed a statistically significant association between having the lowest SpO2 of <70% and pulmonary hypertension (p = 0.016). There were no statistically significant associations between age, gender, smoking status, hypertension, body mass index (BMI), or apnea-hypopnea index (AHI) with occurrence of pulmonary hypertension. AHI is not a good predictor for pulmonary hypertension. The real value of using AHI to predict the health risk of OSA is doubtful. We recommend routine echocardiogram among OSA patient. The objective information in the echocardiogram provides evidence for counseling of patient with disease of OSA and hence hopefully can improve compliance of patient to treatment especially usage of CPAP.

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
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
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*
MiD49 and MiD51: New mediators of mitochondrial fission and novel targets for cardioprotection

Samangouei P, Crespo-Avilan GE, Cabrera-Fuentes H, Hernández-Reséndiz S, Ismail NI, Katwadi KB, et al.

Cond Med, 2018 Aug;1(5):239-246.
PMID: 30338314

Acute myocardial infarction (AMI) and the heart failure (HF) that often follows are among the leading causes of death and disability worldwide. As such novel therapies are needed to reduce myocardial infarct (MI) size, and preserve left ventricular (LV) systolic function in order to reduce the propensity for HF following AMI. Mitochondria are dynamic organelles that can undergo morphological changes by two opposing processes, mitochondrial fusion and fission. Changes in mitochondrial morphology and turnover are a vital part of maintaining mitochondrial health, DNA stability, energy production, calcium homeostasis, cellular division, and differentiation, and disturbances in the balance of fusion and fission can predispose to mitochondrial dysfunction and cell death. Changes in mitochondrial morphology are governed by mitochondrial fusion proteins (Mfn1, Mfn2 and OPA1) and mitochondrial fission proteins (Drp1, hFis1, and Mff). Recent experimental data suggest that mitochondria undergo fission during acute ischemia/reperfusion injury (IRI), generating fragmented dysfunctional mitochondrial and predisposing to cell death. We and others have shown that genetic and pharmacological inhibition of the mitochondrial fission protein Drp1 can protect cardiomyocytes from acute IRI and reduce MI size. Novel components of the mitochondrial fission machinery, mitochondrial dynamics proteins of 49 kDa (MiD49) and mitochondrial dynamics proteins of 51 kDa (MiD51), have been recently described, which have been shown to mediating mitochondrial fission by targeting Drp1 to the mitochondrial surface. In this review article, we provide an overview of MiD49 and MiD51, and highlight their potential as novel therapeutic targets for treating cardiovascular diseases such as AMI, anthracycline cardiomyopathy, and pulmonary arterial hypertension.

Matched MeSH terms: Hypertension, Pulmonary

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