The clinical toxicology of sodium hypochlorite

Introduction: Sodium hypochlorite is used as a bleaching and disinfecting agent and is commonly found in household bleach.

Objective: The objective is to review critically the epidemiology, mechanisms of toxicity, clinical features, diagnosis, and management of hypochlorite poisoning.

Methods: PubMed was searched from January 1950 to June 2018 using the terms “Hypochlorite”, “Sodium Hypochlorite”, “Sodium Oxychloride”, “Hypochlorous Acid”, “Bleach”, “Chlorine Bleach”, in combination with the keywords “poisoning”, “poison”, “toxicity”, “ingestion”, “adverse effects”, “overdose”, and “intoxication”. In addition, bibliographies of identified articles were screened for additional relevant studies including non-indexed reports. Non-peer-reviewed sources were also included. These searches produced 110 citations which were considered relevant.

Epidemiology: There is limited information regarding statistical trends on world-wide poisoning from sodium hypochlorite. In the United States of America, poison control center data have shown that enquiries regarding hypochlorite bleaches have ranged from 43,000 to 46,000 per year over the period 2012–2016.

Mechanisms of toxicity: Hypochlorite’s potential to cause toxicity is related to its oxidizing capacity and the pH of the solution. Toxicity arises from its corrosive activity upon contact with mucous membranes and skin.

Features following ingestion: While small accidental ingestions are very unlikely to cause clinically significant toxicity, large ingestions may cause corrosive gastrointestinal injury and systemic effects, including metabolic acidosis, hypernatremia, and hyperchloremia.

Features following dental exposure: Hypochlorite is used extensively by dentists for cleaning root canals and is safe if the solution remains within the root canal. Extrusions into the periapical area can result in severe pain with localized large and diffuse swelling and hemorrhage.

Features following skin exposure: Prolonged or extensive exposure may cause skin irritation and damage to the skin or dermal hypersensitivity. Such exposures can result in either immediate or delayed-type skin reactions. High concentration solutions have caused severe chemical skin burns.

Features following inhalation: Although there are only limited data, inhalation of hypochlorite alone is likely to lead to no more than mild irritation of the upper airways.

Features following ocular exposure: Corneal injuries from ocular exposure are generally mild with burning discomfort and superficial disturbance of the corneal epithelium with recovery within 1 or 2 days. With higher concentration solutions, severe eye irritation can occur.

Diagnosis: The diagnosis can typically be made on the basis of a careful history, including details of the specific product used, its hypochlorite concentration, and the amount involved. As hypochlorite bleach produces a characteristic smell of chlorine, this may provide a diagnostic clue. In severe cases, corrosive injury is suggested on presentation because of hypersalivation, difficulty swallowing, retrosternal pain or hematemesis.

Management: Symptom-directed supportive care is the mainstay of management. Gastrointestinal decontamination is not beneficial. Local corrosive injury is the major focus of treatment in severe cases. Fiberoptic endoscopy and CT thorax/abdomen are complimentary and have been shown to be useful in corrosive injuries in assessing the severity of injury, risk of mortality and risk of subsequent stricture formation and should be performed as soon as possible after ingestion. Dental periapical extrusion injuries should be left open for some minutes to allow bleeding through the tooth and to limit hematoma development in tissue spaces. Once the bleeding has ceased, the canal can be dressed with non-setting calcium hydroxide and sealed coronally.

Conclusions: Accidental ingestion of household bleach is not normally of clinical significance. However, those who ingest a large amount of a dilute formulation or a high concentration preparation can develop severe, and rarely fatal, corrosive injury so prompt supportive care is essential as there is no specific antidote. Treatment primarily consists of symptom-directed supportive care.     

Treatment needs of drug users: The perspective of Iranian women

Background: Treatment needs and health-care issues in women are completely different from those of men. In order to develop and apply successful treatment programs for substance-dependent women, gender-based differences should be considered to provide them with effective health care and treatment services.

The present study was conducted to investigate the views of substance dependent women about their health care and treatment needs.

Methods: In-depth and semi-structured interviews were conducted among 20 opiate using women who were chosen through purposeful sampling from camps, methadone maintenance treatment (MMT) centers and women’s prison in Sanandaj, Iran in 2015. Data were analyzed using content analysis (a conventional approach).

Findings: Three main factors were recognized through data analysis: individual factors, structural factors, and social factors.

Conclusions: The results of the study suggests the need for structural changes such as improving comprehensive services and enhancing referral system regarding gender-based differences to provide the substance-dependent women with more effective treatment and medical care.     

Exploring an agency-communion model of identity transformation in recovery from substance misuse

Background: Narrative research shows that identity change is key to recovery from substance misuse. Theories have focused on either personal or social factors in this process. A framework encompassing Agency and Communion has been useful in understanding narratives in similar populations.

Objectives: The study proposes that substance misuse and recovery can be understood from an Agency-Communion perspective.

Method: The Life As A Film Task (LAAF) and repertory grids were used to explore Agency and Communion in a sample of 32 participants.

Results: Smallest Space Analysis of LAAF items revealed four different narratives according to themes of Agency and Communion. Case examples indicated that Agency and Communion predicted a recovery identity, and the absence of Agency and Communion predicted substance misuse. Analysis of repertory grids showed fixed low Agency/Communion construct systems in cases of substance misuse and transformed high Agency/Communion construct systems in cases of recovery. Transformation from a low Agency/Communion substance-using identity toward a high Agency/Communion recovery identity was highlighted.

Conclusions: These preliminary findings illustrate the role of Agency and Communion processes in identity-transforming recovery from substance misuse.     

Ask them all: Self-report universal prenatal substance use screening in the United States

Background: Increasing rates of opioid use in the United States have created a national public health crisis. Substance use in pregnancy increases risk for poor birth outcomes. National advisory groups recommend screening all women for prenatal substance use. However, there is no single approach or tool recommended for universal prenatal substance use screening.

Objective: This study seeks to evaluate the effectiveness of universal screening using a direct approach self-report of substance use in pregnancy.

Method: A retrospective review of de-identified data to evaluate women’s willingness to self-report substance use through direct screening. Data obtained from 24 months of maternal self-report were analyzed to assess the overall effectiveness of a direct screening method during the prenatal period.

Results: Findings revealed maternal substance use disclosure rates of 9.7%, higher than the 5.4% United States national average.

Conclusion: Increasing awareness of maternal prenatal substance use offers opportunity for education and support early in pregnancy. Findings suggest the direct method may support maternal self-report of substance use in universal prenatal screening. Additional study with diverse populations and clinical settings is needed.     

Tramadol misuse in the Niger Delta; A review of cases presenting within a year

Objective: Tramadol is an opioid agent used for pain management with huge potentials of misuse. The misuse of Tramadol is associated with increased morbidity and mortality; and this is common among young adults in low-income settings. The aim of this study is to highlight cases of Tramadol misuse managed in a rural teaching hospital in the Niger Delta region of Nigeria.

Method: We used the case report design to highlight five cases of Tramadol misuse in our setting over a year period.

Results: Majority of the cases were males and young adults using Tramadol for non-medical purposes. They all presented with various clinical features including altered levels of consciousness.

Conclusion: Tramadol misuse among young male adults is common in our region. Further research and intervention programs are essential to understand the true burden and also prevent further misuse in the community.     

Mechanisms of toxic cardiomyopathy

Background: Dilated cardiomyopathy is a frequent disease responsible for 40–50% of cases of heart failure. Idiopathic cardiomyopathy is a primary disorder often related to familial/genetic predisposition. Before the diagnosis of idiopathic cardiomyopathy is made, clinicians must not only rule out viral and immune causes, but also toxic causes such as drugs, environmental agents, illicit substances and natural toxins.

Objective: The objective of this review is to present recent data on the mechanisms underlying toxic cardiomyopathy.

Methods: The US National Library of Medicine Pubmed database was searched from 1980 to December 2017 utilizing the combinations of the search terms “toxic cardiomyopathy”, “drugs”, “anticancer drugs”, “azidothymidine”, “rosiglitazone”, “carbon monoxide”, “alcohol”, “illicit drugs”, “cocaine”, “metamfetamine”, “metals”, “venom”. A total of 339 articles were screened and papers that dealt with the pathophysiology of toxic cardiomyopathy, either in animal models or in clinical practice were selected, with preference being given to more recently published papers, which left 92 articles.

Anticancer drugs: The mechanisms of anthracycline-induced cardiotoxicity are primarily related to their mechanisms of action as anticancer drugs, mainly the inhibition of topoisomerase II β and DNA cleavage. Additional metabolic or oxidative stress factors may play a part, together with interference with iron metabolism. The more recent drugs, trastuzumab and imatinib, also influence stress pathways.

Antiretroviral agents: Azidothymidine is cardiotoxic as a result of mitochondrial toxicity. In addition to energy depletion, azidothymidine also increases the production of mitochondrial reactive oxygen species (ROS).

Antidiabetic drugs: The cardiotoxicity of thiazolidinedione antidiabetic drugs is still under investigation, though interference with mitochondrial respiration or oxidative stress is suspected.

Cocaine: Among the multiple mechanisms involved in cocaine-related cardiotoxicity, excessive sympathetic stimulation with increased myocardial oxygen consumption is well documented in the acute form of left ventricular dysfunction. As for cocaine-related cardiomyopathy, the role of apoptosis and ROS is under investigation.

Ethanol: The aetiology of ethanol-related cardiotoxicity is multifactorial, with individual susceptibility being important. It involves apoptosis, alterations of the excitation–contraction coupling in cardiac myocytes, structural and functional alterations of the mitochondria and sarcoplasmic reticulum, changes in cytosolic calcium flows, changes in calcium sensitivity of myofilaments, alterations of mitochondrial oxidation, deregulation of protein synthesis, decrease of contractile proteins and disproportion between the different types of myofibrils, changes in the regulation of myosin ATPase, up-regulation of the L-type calcium channels, increase of oxidative stress, and induction of ANP and p21 mRNA expression in ventricular myocardium.

Metamfetamines: Catecholamine-mediated toxicity is the probable cause, with a possible role for genetic susceptibility.

Carbon monoxide: In addition to hypoxic injury, carbon monoxide is also directly toxic to the mitochondria, with impairment of mitochondrial respiratory chain at the cytochrome c oxidase level, decrease of glutathione concentrations and of ATP production. There is no evidence for a delayed dilated cardiomyopathy in survivors of an acute exposure.

Metals: Cobalt-related cardiomyopathy probably results from interference with energy production and contractile mechanisms, but additional factors (nutrition, hypothyroidism) are often required. Antimony may cause lethal oxidative stress and cell death mediated by elevation in intra-cellular calcium. Proposed mechanisms for mercury toxicity include glutathione depletion, production of ROS, and interruption in selenium-dependent endogenous enzymatic reactions. The existence of a lithium-induced cardiomyopathy is still debated.

Scorpion venom: Catecholamine release is the probable cause of acute cardiomyopathy following scorpion envenomation.

Conclusions: The mechanisms behind toxic cardiomyopathy are complex and multifactorial but include interference with myocardial cell bioenergetics and intracellular calcium handling, the generation of ROS, neurohormonal stress, and induction of apoptosis.     

Need of structural interventions for linking people who inject drugs with antiretroviral treatment: A qualitative study

Objective: People who inject drugs (PWIDs) are highly vulnerable to acquiring HIV infection. Existing interventions have not succeeded in linking PWIDs to antiretroviral treatment (ART). Structural interventions (SIs) or public health interventions that alter the structural context need investigation for a suitable linking to care (LIC) strategy.

Methods: We conducted in-depth interviews with 31 HIV-infected PWIDs. Using a thematic approach, interviews were stopped on reaching saturation of themes.

Results: Personal barriers like denial of HIV status, fatalistic attitude, inability to find time because of earning livelihood, or money for their drugs, transportation difficulties and hospital level barriers like time required for registration, crowd at the hospital, and difficulty traversing departments dissuaded LTC. Using social networks, drug peddlers or provision through mobile health units were accepted as feasible alternatives.

Conclusion: PWIDs face various barriers to LTC. SIs are required for LTC of PWIDs. Storytelling for educational campaign, mobile health units at hot spots to provide HIV test and ART, and using social networks and drug peddlers to assist with education and LTC are suggested.

Practice implications: Existing interventions have not succeeded in reducing HIV prevalence as well as linking to ART care. New SI interventions need to be experimented for LTC.

Abbreviations: AIDS: autoimmune deficiency syndrome; ART: antiretroviral treatment; HIV: human immunodeficiency virus; LTC: linking to care; PWID: people who inject drugs; SI: structural Interventions; TB: htuberculosis Bacillus infection     

The link between childhood trauma and dissociation in frequent users of classic psychedelics and dissociatives

Background: Childhood trauma severity is associated with the level of subsequent substance use as well as with the self-reported severity of dissociation. Classic psychedelics and dissociatives target neurotransmitter systems thought to be involved in the onset of dissociative symptoms and may evoke severe and long-lasting symptoms of depersonalization in some users. However, it is currently unclear whether drug use puts people with a history of childhood trauma at higher risk of developing dissociative symptoms.

Objectives: The current study investigates whether the one-year prevalence of substance use significantly moderates the link between childhood trauma and the severity of depersonalization.

Methods: Participants (n = 297, of which 80.2% were active users) filled out an online self-report questionnaire including the Childhood Trauma Questionnaire (CTQ), the Cambridge Depersonalisation Scale (CDS), and information about their substance use.

Results: Results indicate that childhood trauma and substance use are significant individual predictors of dissociation scores in this sample, but no moderation of substance use on the link between childhood trauma and depersonalization was established.

Conclusions: It is hypothesized that the quality (particularly the context) of the experience of substance use rather than the sheer quantity may be responsible for the manifestation of depersonalization.     

Recommendations to improve opioid use disorder outcomes in countries of the Middle East

Background: Progress has been made in treatment of opioid use disorder (OUD) in the Middle East; current clinical practice often differs from standards of care elsewhere.

Aim: describe treatment approaches in selected countries to inform recommendations for improving care.

Methods: Evidence describing approaches to OUD care was collected and analyzed in a structured, comparative manner. Recommendations were developed based on experts’ clinical experience in the region.

Results: Care differs across countries assessed: Egypt, KSA, UAE, Oman, Kuwait, and Bahrain. Detoxification programs are the common treatment approach in Egypt, KSA, Oman, and Bahrain; integrated programs with opioid agonist therapy (OAT): UAE, Kuwait. Fear of misuse and diversion risk commonly limits access to OAT. Problems with sourcing medicines may limit treatment options. There is limited data on treatment needs or provision. Recommendations: develop effective policy and expert-led consensus on best practice for OUD in the region including integrated treatment programs, provide support for specialists and centers, include innovative medication choices with low diversion risk, promote collaborative work, coordinate data collection, and sharing.

Conclusions: There is important unmet need for OUD in the region and opportunity to improve services through collaboration to support change. Therapy options with reduced diversion risk may address barriers to care.     

The frequency of hospitalizations and length of stay differences between schizophrenic and schizoaffective disorder inpatients who use cannabis

Background: The literature on the effects of cannabis use by patients with schizophrenia is variable.

Aims: To determine whether there are differences among urine-screened cannabis-only users, polysubstance users using cannabis, and nonusers on the number of inpatient hospitalizations and length of stay for patients with schizophrenia or schizoaffective disorder.

Method: This study (N = 116) included patients who tested positive for only cannabis (n = 20, Group 1); tested positive for cannabis and had a history of other substance use (n = 43, Group 2); and had negative drug screens (n = 53, Group 3).

Results: Groups 1 and 2 had significantly lower numbers of hospitalizations than Group 3 without controlling for any other factors, and non-significantly lower lengths of stay than Group 3.

Conclusions: Our results do not support the hypothesis that cannabis exacerbates psychotic symptoms in patients with schizophrenia or schizoaffective disorder. Our study is unique in that it utilized objective urine drug screens.     

Nursing Consultation in a Public Bathhouse: A Community Resource for the Vulnerable Population in a European Capital

Background: In Portugal’s capital, Lisbon, there are 19 public showers (PS) attended by a vulnerable population.

Aim: To describe the assessment and interventions performed during nursing consultations.

Method: This is a quantitative, observational, and cross-sectional study.

Findings: In a convenience sample of 77 users, 52% are females, 43% are single, 52% do not possess any economic resources, 87% have mental health issues, and 32% are homeless. The main nursing focuses found were: arterial hypertension, disease management skills, and emotional suffering. The most used nursing intervention is listening, followed by the expression of feelings, valuing the individual and his/her story, analyzing causes, providing support, and comforting.

Conclusions: This study shows that the main focus of the nursing care in these facilities is the human being. The respect for individuality and dignity was the primary concern, in an attempt to empower the individual to better manage his/her health and illness processes.     

Visit patterns at primary care centres and individual blood pressure level – a cross-sectional study

Objective: Hypertension is a major cause of cardiovascular disease. Nevertheless, blood pressure (BP) is often inadequately treated. We studied visit patterns at primary health care centres (PHCCs) and their relation to individual BP control.

Design and setting: Cross-sectional register-based study on all patients with hypertension who visited 188 PHCCs in a Swedish region.

Patients: A total of 88,945 patients with uncomplicated hypertension age 40–79.

Main outcome measures: Odds ratio (OR) for the individual patient to achieve the BP target of ≤140/90?mmHg.

Results: Overall, 63% of patients had BP?≤?140/90?mmHg (48% BP?<?140/90). The PHCC that the patient was enrolled at and, as part of that, more nurse visits at PHCC level was associated with BP control, adjusted OR 1,10 (95% CI 1.01 to 1.21). Patients visiting PHCCs with the highest proportion of visits with nurses had an even higher chance of achieving the BP target, OR 1.19 (95% CI 1.07 to 1.32).

Conclusions: In a Swedish population of patients with hypertension, about half do not achieve recommended treatment goals. Organisation of PHCC and team care are known as factors influencing BP control. Our results suggests that a larger focus on PHCC organisation including nurse based care could improve hypertension care.     

The availability of raw data in substance abuse scientific journals

Objective: The availability of research data sets is an important milestone since it can enhance the dynamics of research. This study aims to analyse the PubMed Central repository to determine the availability and type of raw data sets in Substance Abuse journals indexed in the Journal Citation Reports.

Methods: A search of the 39 journals from the Substance Abuse category of both editions (Sciences and Social Sciences) of the 2014 Journal Citation Reports was conducted. A study of the supplementary material of the original articles was performed through a search in the PubMed Central repository. The number and types of files were registered.

Results: The search in PubMed Central showed that only 4.7% of original articles contained supplementary material uploaded, being text and .pdf files the most frequent materials whereas spreadsheets and raw data represent less than 10%.

Conclusion: The main finding of this study is that the exchange of data in the substance abuse field is not a common practice since researchers are still reticent.     

Medication adherence,biological and lifestyle risk factors in patients with myocardial infarction: a ten-year follow-up on socially differentiated cardiac rehabilitation

Objective: There is strong evidence that medication adherence and lifestyle changes are essential in patients undergoing secondary cardiovascular disease prevention. Cardiac rehabilitation (CR) increases medication adherence and improves lifestyle changes. Patients with cardiac diseases and a low educational level and patients with little social support are less responsive to improve medication adherence and to adapt lifestyle changes. The aim of the present study was to investigate the long-term effects of a socially differentiated CR intervention on medication adherence as well as changes in biological and lifestyle risk factors at two- five- and ten-year follow-up.

Design: A prospective cohort study.

Setting: The cardiac ward at Aarhus University Hospital, Denmark.

Intervention: A socially differentiated CR intervention in addition to the standard CR program.

Subjects: Patients admitted with first-episode myocardial infarction between 2000 and 2004, N?=?379. Patients were defined as socially vulnerable or non-socially vulnerable according to their educational level and extent of social network.

Main outcome measures: Primary outcome was medication adherence to antithrombotics, beta-blockers, statins and angiotensin-converting enzyme inhibitors. Secondary outcomes were biological and lifestyle risk factors defined as; total cholesterol, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, glycated hemoglobin, blood pressure and smoking status.

Results: No significant long-term effect of the intervention was found.

Conclusions: The results indicate a non-significant effect of the intervention. However, it was found that equality in health was improved in the study population except concerning smoking. General practitioners manage to support the long-term secondary cardiovascular disease prevention in all patients regardless of social status.

• Key points

• The socially differentiated intervention did not significantly improve medication adherence or biological and lifestyle risk factors.

• Despite the non-significant effect of the intervention, equality in health was improved except concerning smoking.

• General practitioners managed to support the long-term secondary cardiovascular disease prevention in all patients regardless of social status.

     

Potential effect of opium addiction on lipid profile and blood glucose concentration in type 2 diabetic patients in Iran

Background: Diabetes is one of the most common chronic diseases in the world. People believe that opium improves blood glucose and lipid profiles in these patients and controversial studies show the effect of consumption of opium in controlling these indices. Accordingly, this study aimed to compare the serum levels of blood indices such as fasting blood glucose (FBS), Hemoglobin A1c (HbA1c) and lipid profile in opium users and non-users in type ΙΙ diabetic patients.

Methods: In this cross-sectional study, among type II diabetic patients referred to the Diabetes Clinic of Birjand 45 opium users and 135 non-users were selected and entered the study by the convenience sampling method.

Results: The results of this study showed that the mean serum levels of FBS, HbA1c, and serum lipid profiles were not significantly different between the two groups of opium users and non-users, but the levels of triglyceride (TG) were significantly 0.18 unit higher in the opium users, compared to non-opium users.

Conclusion: According to the results, the use of opium does not affect the indices of blood glucose, HbA1c and serum lipid profiles except triglyceride in diabetic patients. The general belief that opioid use reduces biochemical indices does not seem to be correct.

Abbreviations: FBS: fasting blood sugar; HbA1c: Hemoglobin A1c; LDL: low-density lipoprotein; HDL: High-density lipoproteins; HDL-C: High-density lipoproteins-cholesterol; BMI: Body mass index; IQR: Inter quartile range; TG: triglyceride; TC: total cholesterol; NS: non-significant; S: significant; ATN: Acute tubular necrosis     

Laparoscopic anterior and posterior native tissue repair: a new pelvic floor approach

Introduction: Traditionally, a cystocele caused by a midline defect of the pelvic fascia is treated by vaginal fascia duplication, also known as anterior colporraphy. The rectocele is managed by suturing the posterior fascia and, frequently, the levator ani muscles. We developed the approach of laparoscopic anterior and posterior fascia repair by native tissue.

Material and methods: The methods were based on anterior and posterior exposure of pelvic fascia similar to the preparation of an extended sacral colpopexy. The fascia was compressed and narrowed by absorbable woven sutures, size 1. Twenty-seven patients were followed up for 6–13?months. All patients received additional apical fixation by pectopexy.

Results: In the examination group, 13 patients underwent anterior laparoscopic fascia repair and 23 had posterior repair. We detected one apical and one posterior relapse, and also one in the anterior repair group. The patient with the apical relapse reported pain and de novo urgency. Anatomical reconstruction was achieved in all other patients.

Summary: Laparoscopic anterior and posterior native tissue repair appears to be a feasible method for the treatment of midline cystocele and rectocele. No new risks were observed. The technique leaves no scar in the vagina and is well accepted.

Abbreviations: POPQ: Pelvic Organ Prolapse Quantification System; FDA: Food and Drug Association; US: United States; Fig: Figure; ICIQ: International Consultation on Incontinence Questionnaire     

Quantitative real-time PCR for differential diagnostics of parvovirus B19 infection in acute liver failure patients

Background: Human Parvovirus B19 (B19V) is a common pathogen worldwide. After primary infection, B19V-DNA may permanently persist in non-erythroid tissues, including the liver of patients with acute liver failure (ALF).

Objective: To validate a real-time PCR (qPCR) for the quantification of B19V-DNA, in order to establish a differential diagnosis for B19V infection in ALF patients.

Methods: The qPCR techniques were based on Sybr Green® and TaqMan® methodologies. To evaluate the quality parameters of both methods, samples from patients with or without B19V infection were tested. The diagnostic utility of qPCR in the detection B19V-DNA in patients with ALF was evaluated by testing archived serum and hepatic tissue explants from 10 patients.

Results: The Sybr Green® methodology showed 97% efficiency, the limits of detection and quantification were 62.6 and 53,200 copies/mL, respectively. The TaqMan® methodology showed 95% efficiency, the limits of detection and quantification were 4.48 and 310 copies/mL, respectively. A false positive result was found only with the Sybr Green® methodology. Among ALF patients without defined etiology, three (30%) were positive for B19V DNA in serum and liver.

Conclusion: The qPCR methods validated here were effective in clarifying uncommon cases of B19V-related ALF and are fit for differential diagnosis of ALF causes.     

Diagnosis and treatment of pediatric pulmonary arterial hypertension

Introduction: Pediatric pulmonary arterial hypertension (PAH) remains a rare and severe disease with a poor prognosis. PAH may be idiopathic, heritable or associated with systemic conditions in particular associated with congenital heart disease.

Areas covered: A thorough and extensive diagnostic approach is required for a correct diagnosis. The outcome has improved over the last decade with a better diagnostic approach and with the initiation of new targeted therapies. However, there is still significant progress to achieve as there is still no cure for this devastating disease.

Expert opinion: Adapted clinical studies to define the best therapeutic approach are needed. Even if the treatment approach is still mainly derived from adult data and expert consensus, several studies and registries are currently underway and should deliver important information in the next future.

This review aims to give an overview of the current diagnosis and treatment strategies of PAH.