Pulmonary hypertension (PH) is an end result of a diverse array of complex clinical conditions that invoke hemodynamic and pathophysiological changes in the pulmonary vasculature. Many patients’ symptoms begin with dyspnea on exertion for which screening tests such as chest roentgenograms and more definitive noninvasive tests such as CT scans are ordered initially. It is imperative that clinicians are cognizant of subtle clues on these imaging modalities that alert them to the possibility of PH. These clues may serve as a stepping stone towards more advanced noninvasive (echocardiogram) and invasive (right heart catheterization) testing. On the CT scan, the signs are classified into mediastinal and lung parenchymal abnormalities. In addition to suspecting the diagnosis of PH, this paper provides a pictorial essay to guide health care professionals in identifying the etiology of PH. This paper also provides concrete definitions, wherever possible, of what constitutes abnormalities in PH, such as dilated pulmonary arteries, pruning of vessels, and increased thickness of free wall of the right ventricle. The sensitivities and specificities of each sign are enumerated. The common radiographic and clinical features of many different etiologies of PH are tabulated for the convenience of the readers. Some newer imaging modalities such as dual-energy CT of the chest that hold promise for the future are also described.
In this trial, 261 patients with chronic thromboembolic pulmonary hypertension were assigned to placebo or to the soluble guanylate cyclase stimulator riociguat. At 16 weeks, riociguat had significantly improved the 6-minute walk distance and pulmonary vascular resistance. Chronic thromboembolic pulmonary hypertension is characterized by obstruction of the pulmonary vasculature by residual organized thrombi, 1 leading to increased pulmonary vascular resistance, progressive pulmonary hypertension, and right ventricular failure. 2 , 3 Patients with chronic thromboembolic pulmonary hypertension have a poor prognosis unless they receive treatment early. 4 Pulmonary endarterectomy is the standard treatment for chronic thromboembolic pulmonary hypertension and is the only potentially curative treatment. 5 However, surgery is not an option for all patients; some patients are ineligible for surgery owing to the occlusion of distal vessels or coexisting conditions, some decline surgery, and some do not have access to expert surgical . . .
SUMMARY POINTS Pulmonary hypertension has many causes so prognoses and treatments vary The condition is diagnosed by systematically evaluating the breathless patient and screening patients at high risk Patients at high risk of severe and treatable pulmonary hypertension include those with systemic sclerosis, portal hypertension, congenital heart disease, and previous pulmonary embolism Specialist centres provide access to tailored investigative and treatment pathways and support networks for patients Patients with severe pulmonary hypertension can deteriorate rapidly—do not delay referral to perform specialist investigations Only selected patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension benefit from interventions directed at pulmonary vasculature; for most patients treatment is aimed at the underlying condition
Patients with sickle cell disease underwent ECG with assessment of tricuspid valve regurgitant jet velocity (TRJV) to screen for pulmonary hypertension, followed by right heart catheterization if the TRJV was 2.5 m per second or more. Prevalence was 27% on the basis of ECG criteria but only 6% on the basis of catheterization. In several studies, pulmonary hypertension, particularly pulmonary arterial hypertension, has been reported as a frequent complication of sickle cell disease. 1 – 4 Pulmonary arterial hypertension is characterized by the presence of precapillary pulmonary hypertension in the absence of left-sided heart disease, lung disease, or chronic thromboembolism. On histopathological analysis, pulmonary arterial hypertension is characterized by the proliferation of medial smooth-muscle cells and endothelial cells in the small pulmonary arteries. 5 Pulmonary arterial hypertension may be idiopathic, heritable, or associated with other disorders, such as connective tissue diseases and congenital heart disease. 6 In the updated classification of pulmonary hypertension, sickle cell disease appears . . .
The number of effective, long-term treatments for pulmonary hypertension is limited. In this double-blind, randomized trial, an aerosolized form of iloprost, a stable analogue of the pulmonary vasodilator prostacyclin, was assessed over a 12-week period. Iloprost had a beneficial effect on the combined end point of the distance walked in six minutes and an improvement in the New York Heart Association functional class. A continuous infusion of prostacyclin was the first therapy shown to reduce mortality in a controlled study of patients with severe pulmonary hypertension. 1 However, its use is associated with a number of serious drawbacks. The lack of pulmonary selectivity results in systemic side effects, tolerance leads to progressive increases in the dose, and there may be recurrent infections of the intravenous catheter. 2 As an alternative, inhaled nitric oxide possesses pulmonary selectivity, but it is less potent than prostacyclin in the pulmonary vasculature. 3 , 4 Moreover, an interruption in the inhalation of continuous nitric oxide may cause rebound pulmonary hypertension. 5 , 6 Designed . . .
Summary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m−2 ). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies. Funding Actelion Pharmaceuticals.