[Pulmonary hypertension associated with left heart disease (group 2)]. / Pulmonale Hypertonie assoziiert mit Linksherzerkrankungen (Gruppe 2).

Pulmonary hypertension associated with left heart disease (PH-LHD) corresponds to group two of pulmonary hypertension according to clinical classification. Haemodynamically, this group includes isolated post-capillary pulmonary hypertension (IpcPH) and combined post- and pre-capillary pulmonary hypertension (CpcPH). PH-LHD is defined by an mPAP > 20 mmHg and a PAWP > 15 mmHg, pulmonary vascular resistance (PVR) with a cut-off value of 2 Wood Units (WU) is used to differentiate between IpcPH and CpcPH. A PVR greater than 5 WU indicates a dominant precapillary component. PH-LHD is the most common form of pulmonary hypertension, the leading cause being left heart failure with preserved (HFpEF) or reduced ejection fraction (HFmrEF, HFrEF), valvular heart disease and, less commonly, congenital heart disease. The presence of pulmonary hypertension is associated with increased symptom burden and poorer outcome across the spectrum of left heart disease. Differentiating between group 1 pulmonary hypertension with cardiac comorbidities and PH-LHD, especially due to HFpEF, is a particular challenge. Therapeutically, no general recommendation for the use of PDE5 inhibitors in HFpEF-associated CpcPH can be made at this time. There is currently no reliable rationale for the use of PAH drugs in IpcPH, nor is therapy with endothelin receptor antagonists or prostacyclin analogues recommended for all forms of PH-LHD.

Pulmonary hypertension associated with left heart disease: Updated Recommendations of the Cologne Consensus Conference 2018.

In the summer of 2016, delegates from the German Society of Cardiology (DGK), the German Respiratory Society (DGP), and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH associated with left heart disease. In this context, the European Guidelines point out that the drugs currently approved to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, sGC stimulators) have not sufficiently been investigated in other forms of PH. However, despite the lack of respective efficacy data, an uncritical use of targeted PAH drugs in patients with PH associated with left heart disease is currently observed at an increasing rate. This development is a matter of concern. On the other hand, PH is a frequent problem that is highly relevant for morbidity and mortality in patients with left heart disease. In that sense, the distinction between isolated post-capillary pulmonary hypertension (IpcPH) and combined post- and pre-capillary pulmonary hypertension (CpcPH) and their proper definition may be of particular relevance. The detailed results and recommendations of the working group on PH associated with left heart disease, which were last updated in the spring of 2018, are summarized in this article.

Endomyocardial biopsy in patients with cardiomyopathy of unknown origin: does specialized center experience apply to a tertiary care hospital?

BACKGROUND: In patients with cardiomyopathy of unknown origin, endomyocardial biopsy provides the possibility of improved diagnosis and tailored treatment. Specific guidance has been developed based on cardiovascular centre of excellence experience but it is unknown if the benefits also extend into the tertiary care hospital setting. METHODS: Endomyocardial biopsies was performed in patients with cardiomyopathy of unknown origin. The outcomes were mirrored against the current ESC recommendations. RESULTS: A total of 57 patients with cardiomyopathy of unknown origin underwent endomyocardial biopsy with a mean age of 54 years and 28 % being women. In 17 patients (30 %), viruses were detected in the biopsy material, in 6 patients (11 %) cardiac amyloidosis was found of which 3 had also a positive test for viruses. The overall mortality rate was 18 % in the mean follow up period of 30 months, with a rate of 24 % in those with virus detection (mean FU 24 months) and 15 % in those without virus detection (mean FU 31 months. Death rates were 83 % in patients with cardiac amyloidosis (mean FU 10 months). CONCLUSION: We conclude that, limited by uncertainty stemming from the small number of included patients, endomyocardial biopsy may not prove to have a clinical impact on treatment decisions and outcomes in a tertiary care hospital setting. We consider cardiac amyloidosis to be an exception, since the mortality rate with or without concomitant virus load was extremely high.

Pulmonary hypertension due to left heart disease: updated Recommendations of the Cologne Consensus Conference 2011.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more frequent forms of PH including PH owing to left heart disease. The guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) have not been sufficiently investigated in other forms of PH. However, despite the lack of respective efficacy data an uncritical use of targeted PAH drugs in patients with PH associated with left heart disease is currently observed at an increasing rate. This development is a matter of concern. On the other hand, PH is a frequent problem that is highly relevant for morbidity and mortality in patients with left heart disease. It that sense, the practical implementation of the European Guidelines in Germany requires the consideration of several specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH due to left heart disease. This commentary describes in detail the results and recommendations of the working group which were last updated in October 2011.

[Complication of a complex heart failure: perforation of a sinus Valsalva [corrected] aneurysm]. / Therapiekomplikation einer komplizierten Herzinsuffizienz: perforiertes Sinus-Valsalvae-Aneurysma.

A 68-year-old woman was admitted to hospital because of chronic heart failure. The cause was a left-right shunt between the left sinus of Valsalva and the right atrium. The patient underwent surgery with shunt closure. Intraoperatively, the ramus circumflexus of the coronary artery was occluded. 2.5 years later the patient was readmitted to hospital. There was acute heart failure caused by an ischemic mitral regurgitation III degrees . A planned mitral valve replacement and operative myocardial revascularization were not performed because the patient developed a drug-induced hepatotoxic failure under phenprocoumon.