Invasive Haemodynamic Assessment Before and After Left Ventricular Assist Device Implantation: A Guide to Current Practice

Mechanical circulatory support for the management of advanced heart failure is a rapidly evolving field. The number of durable long-term left ventricular assist device (LVAD) implantations increases each year, either as a bridge to heart transplantation or as a stand-alone ‘destination therapy’ to improve quantity and quality of life for people with end-stage heart failure. Advances in cardiac imaging and non-invasive assessment of cardiac function have resulted in a diminished role for right heart catheterisation (RHC) in general cardiology practice; however, it remains an essential tool in the evaluation of potential LVAD recipients, and in their long-term management. In this review, the authors discuss practical aspects of performing RHC and potential complications. They describe the haemodynamic markers associated with a poor prognosis in patients with left ventricular systolic dysfunction and evaluate the measures of right ventricular (RV) function that predict risk of RV failure following LVAD implantation. They also discuss the value of RHC in the perioperative period; when monitoring for longer term complications; and in the assessment of potential left ventricular recovery.

Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood

Idiopathic pulmonary arterial hypertension (IPAH) is a rare but fatal disease diagnosed by right heart catheterisation and the exclusion of other forms of pulmonary arterial hypertension, producing a heterogeneous population with varied treatment response. Here we show unsupervised machine learning identification of three major patient subgroups that account for 92% of the cohort, each with unique whole blood transcriptomic and clinical feature signatures. These subgroups are associated with poor, moderate, and good prognosis. The poor prognosis subgroup is associated with upregulation of the ALAS2 and downregulation of several immunoglobulin genes, while the good prognosis subgroup is defined by upregulation of the bone morphogenetic protein signalling regulator NOG, and the C/C variant of HLA-DPA1/DPB1 (independently associated with survival). These findings independently validated provide evidence for the existence of 3 major subgroups (endophenotypes) within the IPAH classification, could improve risk stratification and provide molecular insights into the pathogenesis of IPAH.

Reduction of Pulmonary Hypertension After Transition to Sacubitril/Valsartan in Patients With Heart Failure With Preserved Ejection Fraction

Objectives: Although the PARAGON-HF trial failed to reach its primary endpoint, subgroups of patients with heart failure with preserved ejection fraction (HFpEF) still appear to benefit from Sacubitril/Valsartan therapy. As HFpEF patients with pulmonary hypertension display a specifically high mortality and morbidity, we evaluated the effect of Sacubitril/Valsartan in this subgroup of HFpEF patients.Methods: In this retrospective case-series of 18 patients with HFpEF and pulmonary hypertension, right heart catheterisation (RHC) for determination of invasive pulmonary pressure were performed at baseline (pre-Sacubitril/Valsartan) and 99 (71–156) days after transition from angiotensin-converting enzyme inhibitors and angiotensin receptor blockers to Sacubitril/Valsartan (post-Sacubitril/Valsartan). Results are given as median and interquartile range.Results: After conversion to Sacubitril/Valsartan, RHC showed significantly reduced pulmonary artery pressure (PAP) and mean pulmonary capillary wedge pressure (PCWP) compared to pre-Sacubitril/Valsartan [PAP systolic/diastolic/mean 44 (38–55)/15 (11–20)/27 (23–33) mm Hg vs. 51 (41–82)/22 (13–29)/33 (28–52) mm Hg, p < 0.05 and p < 0.01, respectively; PCWP 16 (12–20) mm Hg vs. 22 (15–27) mm Hg, p < 0.05]. Median Sacubitril/Valsartan dosage was 24/26 mg BID (24/26 BID−49/51 mg BID). Clinically, New York Heart Association functional class improved in 12 of the 18 patients (p < 0.01) after conversion to Sacubitril/Valsartan. Echocardiographic parameters of left ventricular function and cardiovascular co-medication did not differ markedly between pre- and post-Sacubitril/Valsartan.Conclusion: Sacubitril/Valsartan therapy is associated with an improvement of pulmonary hypertension in HFpEF patients.

Ventricular–vascular coupling is predictive of adverse clinical outcome in paediatric pulmonary arterial hypertension

AimsVentricular–vascular coupling, the ratio between the right ventricle’s contractile state (Ees) and its afterload (Ea), may be a useful metric in the management of paediatric pulmonary arterial hypertension (PAH). In this study we assess the prognostic capacity of the ventricular–vascular coupling ratio (Ees/Ea) derived using right ventricular (RV) pressure alone in children with PAH.MethodsOne hundred and thirty paediatric patients who were diagnosed with PAH via right heart catheterisation were retrospectively reviewed over a 10-year period. Maximum RV isovolumic pressure and end-systolic pressure were estimated using two single-beat methods from Takeuchi et al (Ees/Ea_(Takeuchi)) and from Kind et al (Ees/Ea_(Kind)) and used with an estimate of end-systolic pressure to compute ventricular–vascular coupling from pressure alone. Patients were identified as either idiopathic/hereditary PAH or associated PAH (IPAH/HPAH and APAH, respectively). Haemodynamic data, clinical functional class and clinical worsening outcomes—separated into soft (mild) and hard (severe) event categories—were assessed. Adverse soft events included functional class worsening, syncopal event, hospitalisation due to a proportional hazard-related event and haemoptysis. Hard events included death, transplantation, initiation of prostanoid therapy and hospitalisation for atrial septostomy and Pott’s shunt. Cox proportional hazard modelling was used to assess whether Ees/Ea was predictive of time-to-event.ResultsIn patients with IPAH/HPAH, Ees/Ea_(Kind) and Ees/Ea_(Takeuchi) were both independently associated with time to hard event (p=0.003 and p=0.001, respectively) and when adjusted for indexed pulmonary vascular resistance (p=0.032 and p=0.013, respectively). Neither Ees/Ea_(Kind) nor Ees/Ea_(Takeuchi) were associated with time to soft event. In patients with APAH, neither Ees/Ea_(Kind) nor Ees/Ea_(Takeuchi) were associated with time to hard event or soft event.ConclusionsEes/Ea derived from pressure alone is a strong independent predictor of adverse outcome and could be a potential powerful prognostic tool for paediatric PAH.

Whooping cough complicating pulmonary hypertension in pregnancy

We report a case of a previously fit woman who presented at 26 weeks into her fourth pregnancy with a dry cough. Following a nasopharyngeal swab, she was diagnosed with a pertussis infection, and treated with antibiotics. A chest X-ray showed right atrial dilatation and an echocardiogram was scheduled outpatient. However, after re-presenting with worsening cough and dyspnoea, an inpatient echocardiogram was performed which suggested elevated pulmonary pressures with significant tricuspid regurgitation, as confirmed by subsequent cardiac catheterisation. She had an elective caesarean section at 34 weeks and underwent repeat right heart catheterisation which revealed persistent, and likely pre-existing, pulmonary arterial hypertension. This case highlights the importance of thorough assessment of non-obstetric symptoms in pregnancy in formulating alternative differentials, even after a diagnosis has been made, to prevent potentially life-threatening conditions from being missed. It also shows that although often associated, respiratory and cardiac causes may coexist separately.

Current strategies for managing CTEPH: results of the worldwide prospective CTEPH Registry

BackgroundPulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy, and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH registry identifies clinical characteristics of patients, diagnostic algorithms, and treatment decisions in a global context.Methods1010 newly diagnosed consecutive patients were included into the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography, and/or invasive pulmonary angiography after at least 3 months on anticoagulation.ResultsOverall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability: technical inaccessibility (n=235), co-morbidities (n=63), and patient refusal (n=44). In Europe, America and other countries (AAO), 72% of patients were deemed suitable for PEA whereas in Japan, 70% of patients were offered BPA as first choice. Gender was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates, and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of PH-related causes (3.5% after PEA and 1.8% after BPA).ConclusionsThe registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and gender) and therapeutic approaches in Japan compared with Europe and AAO.

Wide pulse pressure and Quincke’s pulse in high-output heart failure

A 74-year-old man with a history of chronic alcohol use presented with progressive exertional dyspnoea and weight gain. On physical examination, he was noted to have wide pulse pressure, elevated jugular venous pressure, and alternating flushing and blanching of the nail beds in concert with the cardiac cycle, known as Quincke’s pulse. Transthoracic echocardiography demonstrated normal biventricular systolic function and valvular function, but noted a dilated inferior vena cava. Right heart catheterisation revealed elevated filling pressures, high cardiac output and low systemic vascular resistance, consistent with high-output heart failure. Whole blood concentration of thiamine was low, confirming the diagnosis of wet beriberi. The patient abstained from alcohol use and was started on thiamine replacement therapy, resulting in narrowing of the pulse pressure over time and complete resolution of symptoms without the need for diuretic therapy.

Chronic thromboembolic pulmonary hypertension: a review

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension resulting from obstruction of the pulmonary artery by fibrotic thromboembolic material, usually initiated by recurrent or incomplete resolution of pulmonary embolism. This distinct form of pulmonary hypertension is classified under Group 4 of the World Health Organization classification. Further investigations are usually initiated, with transthoracic echocardiography followed by right heart catheterisation and pulmonary angiography as the gold standard. Definitive treatment is usually in the form of surgical pulmonary endarterectomy. Inoperable CTEPH is medically treated with pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators and prostacyclin. Recent developments have made balloon pulmonary angioplasty a viable option as well.