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Cardiovascular system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
The murmur of atrial septal defect is not present at birth. It is due to increased flow across the pulmonary outflow tract and valve. The fixed splitting is due to constantly increased right ventricular diastolic volume and prolonged ejection time. The increased blood flow to the lungs is seen as pulmonary plethora on X-ray. Pulmonary hypertension can result from the increased blood flow to the lung.
Unusual Inherited Pulmonary Diseases Which Provide Clues to Pulmonary Physiology and Function
Published in Stephen D. Litwin, Genetic Determinants of Pulmonary Disease, 2020
Thomas Κ. C. King, Robert A. Norum
Symptoms usually appear when the pulmonary hypertension is well established. Dyspnea on exertion, fatigue, and weakness are related to a low cardiac output which cannot increase on demand. Similarly, syncope may occur on effort or, when occurring at rest, is likely to be due to cardiac arrhythmias. Chest pain on exertion is probably the result of ischemia of the considerably hypertrophied right ventricle. Hoarseness may result from compression of the left recurrent laryngeal nerve by the enlarged left pulmonary artery. Hemoptysis is not uncommon, usually minor, but may be recurrent. Sudden death is a serious risk and is particularly liable to occur during catheterization, anesthesia, or surgical procedure. The mechanism is either arrhythmias or acute right ventricular failure.
Common cardiac conditions, drugs and methods of assessment
Published in Judy Bothamley, Maureen Boyle, Medical Conditions Affecting Pregnancy and Childbirth, 2020
Pulmonary hypertension is a progressive haemodynamic condition characterised by a rise in the blood pressure of the pulmonary arteries. It is not strictly congenital heart disease but is discussed together with Eisenmenger’s because it has a similarly serious nature in pregnancy. The cause of pulmonary hypertension may be unknown (idiopathic) or secondary to a number of conditions, including congenital heart disease, HIV drugs and other diseases such as haemoglobinopathies or thyroid disorders. It may also be associated with some lung diseases or thrombotic or embolic disease (Parambil and McGoon, 2007).
Strategies for optimizing intravenous prostacyclin-analog therapy in patients with pulmonary arterial hypertension
Published in Expert Review of Respiratory Medicine, 2022
Ralf Ewert, Dirk Habedank, Michael Halank, Beate Stubbe, Christian F. Opitz
For many years, PAH therapy has been focused on meeting certain goals, with hemodynamic parameters and functional class being established as relevant factors in prognosis [60,61]. This principle of goal-directed therapy regarding prognostic factors has been adopted in the selection of clinical endpoints in randomized studies of new therapies for PAH [62]. The increasing evidence (by registries [63] and other studies) on risk factors in PAH patients was summarized in a statement by the ERS/ESC on the risk assessment in PH [64]. This proceeding and the selected risk factors have been validated in recent years [65], with a recent switch to favoring consolidation of these factors focusing on noninvasive parameters [66–68]. The last World Conference on Pulmonary Hypertension confirmed this stepwise therapeutic approach based on the patient´s individual risk [69]. Along these lines, an initial combination therapy including SC and IV PCA is highly recommended, especially for patients at high risk. The enormous potential of such a combination therapy has been shown in 19 PH patients with severe hemodynamic impairment at the time of first diagnosis [70]. These patients were treated up-front with progressively increasing doses of sildenafil, bosentan, and EP over a few weeks (target dose after 4 months, 16 ng/kg/min), with a survival rate of 100% after a mean follow-up of 41 ± 13 months.
Prognostic value of main pulmonary artery diameter to ascending aorta diameter ratio in patients with advanced heart failure
Published in Acta Cardiologica, 2021
Cem Dogan, Zubeyde Bayram, Süleyman Cagan Efe, Rezzan Deniz Acar, Ibrahim Halil Tanboga, Ali Karagoz, Nuri Havan, Tanıl Ozer, Abdulkair Uslu, Mehmet Kaan Kırali, Cihangir Kaymaz, Nihal Ozdemir
The relationship between dilatation of pulmonary artery and pulmonary hypertension is well established. For HT candidates, the prevalence of PH due to left heart disease (PH-LHD) ranges from 12% to 38% and it may result with right ventricle failure after HT [10–12]. Echocardiography is commonly used as non-invasive and practical method for diagnosing PH-LHD. A recent meta-analysis suggested that echocardiography was considered as an accurate approach in estimating the PAPs of 63% of the patients, with 56% specificity [13]. However, RHC remains the gold standard technique to evaluate pulmonary vascular pressure and is therefore recommended in patients considered candidates for heart transplantation [7–12]. Recently, CT scan has become a commonly used technique to assess pulmonary vascular diseases. The rational of using CT scan in the diagnostic process of pulmonary vascular disease is; to determine the dilatation of pulmonary artery and its’ branches developed secondary to PH. The increased size of these vessels can be attributed to the changes by vascular remodelling, which is influenced by the blood flow, vascular compliance, peripheral resistance, pathology within the vessel, sex and body constitution [14,15].
New promising drugs for the treatment of systemic sclerosis: pathogenic considerations, enhanced classifications, and personalized medicine
Published in Expert Opinion on Investigational Drugs, 2021
Alain Lescoat, John Varga, Marco Matucci-Cerinic, Dinesh Khanna
Recent breakthroughs in the management of pulmonary hypertension, with a positive impact on survival, have been achieved by early association of existing drugs rather than the design of new treatments. This should inform the research on investigational drugs in SSc, especially considering that immunomodulatory drugs are now broadly used in patients with early dcSSc. Therefore, the identification of new therapeutic options has to be considered on top of existing immunomodulators already in use as background therapies. The association of monoclonal antibodies could also constitute a change of paradigm in the management if SSc, if such combinations prove to be well tolerated. The simultaneous inhibition of profibrotic and pro-inflammatory pathways by a single drug (JAK inhibitors) or through combination strategies (pirfenidone and MMF, or romilkimab and immunomodulators) may constitute a promising option for disease-modifying management of SSc. The introduction of anti-fibrotic drugs at the early inflammatory phase of the disease, in addition to immunomodulatory treatments, may also allow clinically meaningful results for patients with dcSSc in the future. Nonetheless, the relevance and safety of such associations are still to be determined in RCT.