Katarzyna Małaczyńska-Rajpold

The Management of Supraventricular Tachyarrhythmias in Patients with Pulmonary Arterial Hypertension.

BACKGROUND Atrial remodelling in pulmonary arterial hypertension (PAH) may lead to higher incidence of supraventricular arrhythmias (SVA). The purpose of this study was to evaluate the efficiency and safety of various methods for treatment of SVA in this group. METHODS This was a single centre study. Forty-eight patients (33 women and 15 men) aged 19-77 years (median 49 years) were enrolled. There were 30 patients with idiopathic PAH, 10 had PAH associated with connective tissue disease, and eight with congenital heart disease. A retrospective analysis was performed to estimate the prevalence and type of supraventricular arrhythmias, as well as efficiency and safety of treatment methods. Mean follow-up period was 28.8±17.7 months. RESULTS Supraventricular arrhythmias occurred in 17 patients (35%) and appeared to be atrial fibrillation, flutter or tachycardia. Supraventricular arrhythmias coexisted with elevated mean right atrial pressure in 75%. Four patients had more than one type of SVA. A flutter-like macro-reentrant form of atrial tachycardia dependent on cavo-tricuspid isthmus was found in four cases. The treatment of SVA included typical methods: antiarrhythmic drugs, direct current cardioversion (DCC), and radiofrequency (RF) ablation. All of the therapeutic methods were effective in managing acute arrhythmia. Three patients required re-ablation. Overall mortality: 14 patients (29%) in the whole study group, including six in SVA group (35%) and eight without SVA (26%). CONCLUSIONS In patients with PAH DCC, pharmacological cardioversion and RF ablation can be applied safely and effectively. Flutter-like macro-reentrant atrial tachycardia dependent on cavo-tricuspid isthmus is observed in this group. It is more challenging, but possible, to successfully treat this arrhythmia with RF ablation.

Red Blood Cells Distribution Width as a Potential Prognostic Biomarker in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

BACKGROUND Red blood cells distribution width (RDW) predicts survival in cardiovascular diseases. Little is known about the variability of RDW level over time among patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). To our knowledge, RDW has never been analysed as a marker of response to specific treatment. MATERIALS AND METHODS We retrospectively analysed 77 patients for: i) RDW measured during the last hospitalisation before death or during the last follow-up (RDWlast); ii) mean RDW from all hospitalisations during the entire follow-up of the patient (RDWmean); iii) maximum RDW of all hospitalisations of each patient (RDWmax). In order to assess response to specific treatment and association with prognosis, we compared RDW levels (obtained from 56 patients) before and 3 to 6 months after introduction or intensification of treatment in both the alive and deceased group. RESULTS Twenty-eight of 77 patients died, whereas in specific drugs treatment response analysis, 22 of 56 patients died during follow-up. The cut-off values derived from the ROC analysis and assessed using the log-rank test were significant for RDWlast (p<0.0001), RDWmean (p<0.001) and RDWmax (p=0.02). A decrease in RDW levels after introduction or intensification of specific treatment was significant (p=0.015) in survivors, whereas there was no significance (p=0.29) in decrease in RDW levels in non-survivors after change of therapy. CONCLUSIONS Red blood cells distribution width might be a potential prognostic biomarker in patients with PAH and inoperable CTEPH. The decrease in RDW level after introduction or escalation of PAH-targeted and CTEPH-targeted drugs is associated with a good treatment response and better prognosis.

The "bouncing" catheter.

Treprostinil sodium, as a prostacyclin analogue, is a specific drug used for treatment of pulmonary arterial hypertension (PAH) [1]. One way of administration — intravenous — may be provided by implantation of a subcutaneous pump (LenusPro) in the subcostal region. It administers the drug continuously to the superior caval vein by means of a catheter inserted through the subclavian vein [2–4]. This method is generally safe and adverse events occur rarely [5]. A 38-year-old man with PAH treated with treprostinil had a LenusPro pump (Fig. 1A, E) implanted. The chest X-ray after 2 months revealed the tip of the catheter placed in the right internal jugular vein (Fig. 1B). As the patient had been in a good clinical condition, we decided to observe it. Four months after implantation the patient reported a strong pain and swelling in the right subclavian region without significant clinical deterioration. The chest X-ray revealed the tip of the catheter below the right clavicle (Fig. 1C) — the catheter slipped out of the vascular system and the drug was being delivered into the soft tissues. Due to tissue edema, the repositioning of the catheter attempted in the implanting center was difficult and required carrying the cannula through the supraclavicular region into the right internal jugular vein (Fig. 1D). After next 2 months, while checking the position of the catheter in the fluoroscopy, we suspected another dislocation (Fig. 1F). On closer assessment in the implanting center, the cannula was seen to be folding and straightening in the jugular vein broadened due to chronically elevated pressure (Fig. 1G, H). This time the cannula apparently remained within the vascular lumen and those moves did not result in clinical deterioration of the patient’s condition during several months of follow-up. However, the residual volume in the pump had been higher than expected and finally the outflow stopped, giving an alarm. This time the patient required an urgent repositioning of the catheter, which were placed again through the proximal subclavian vein, whereas the distal part appeared to be occluded with well-developed collateral circulation. In patients with PAH and a LenusPro pump for treprostinil infusion, there is a possibility for catheter dislocation. It is reasonable to check the position of the catheter regularly with an imaging method (i.e. chest X-ray, fluoroscopy). A strong pain in the subclavian region may suggest that the cannula is slipping out of the subclavian vein. In the presented case, such a situation required urgent repositioning of the catheter due to the pain and worse absorption of the drug from the soft tissues. An unstable position of the tip of the cannula without its slipping outside the vessel also does not appear to be benign, because one day it may result in complete wedging of the catheter with an outflow blockade.

Balloon atrial septostomy in pulmonary arterial hypertension: A beneficial effect on the control of rhythm abnormalities.

Balloon atrial septostomy (BAS) is a palliative procedure, but actually, interatrial shunt tends to be self-closing within a few months. However, in countries with a large number of donors, time to lung transplantation (LuTx) for a patient on a waiting list it is approximately 2–4 months [2]. Such a short waiting time allows the procedure to be performed only once or even offers the possibility of extracorporeal membrane oxygenation if the patient requires bridging treatment [3]. In cases with an essentially longer waiting list, there is a need for methods which allow a deteriorating patient to survive for a year or even up to 2 years. Using one of the devices available on the market in addition to BAS, to keep the interatrial connection opened within this time period, prevents the patient from having the procedure repeated. These devices are very promising — especially the atrial flow regulator (AFR) invented by Vettukattil’s group [4]. However, currently, some significant disadvantages exist comprising: i) the limited availability for commercial use, ii) high price and iii) lack of reimbursement for the procedure. Patients disqualified from LuTx would probably benefit significantly from the implantation of such a device as their life would be prolonged without the need for repeated invasive BAS procedure [5]. On the other hand, however, there are patients referred for LuTx for which another question arises: what to do with such devices in patients undergoing LuTx. The only device which is retrievable is the AFR. However, after a period of implantation, the device gets endothelialized and retrieval becomes impossible. Thus, the only remaining option seems to be an AFR closure with an atrial septal occluder or vascular plug. But when? During transplantation or some time later? Perhaps such a patent connection between the atria could be helpful in the initial period after LuTx, because the flow direction in the shunt would reverse, causing a bigger preload and in this way improving its function before adapting to the lower vascular resistance of the new lungs. The current ongoing multicenter trial in which the AFR device is being tested will perhaps tell us more precisely, how to face these challenges.

Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Background: Upregulation of the immune system is regarded to play an important role in the etiopathobiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). To the best of our knowledge, neopterin (NP) has never been investigated in patients with PAH and CTEPH. Objectives: The aim of the study was to evaluate the concentration of NP in blood in order to examine its impact on outcome and relationship with disease severity in that population. Methods: Serum concentration of NP was analysed prospectively in 50 patients (36 with PAH and 14 with CTEPH vs. 31 healthy controls) and assessed in relation to clinical parameters and outcome. Results: NP concentration in the PAH and CTEPH groups combined was significantly higher than in the control group (8.68, 6.39–15.03 vs. 5.14, 4.16–5.98 nmol/L, p < 0.0000001). During 9 months of follow-up, clinical deterioration occurred in 18 patients (including 8 deaths), and NP concentration in this group was higher when compared to stable patients (15.6, 8.52–25.13 vs. 7.87, 6.18–9.89, p = 0.002). The cutoff value of NP derived from ROC curve analysis was 15.3 nmol/L (p = 0.002, AUC 0.77, p = 0.0004, HR = 4.35, 95% CI 1.43–13.18, log-rank test). On Cox regression analysis, NP predicted clinical deterioration (p = 0.009, 95% CI 1.01–1.06). NP correlated positively with NT-proBNP (p < 0.001), red blood cell distribution width (p < 0.001), and right atrium area (p = 0.002) and inversely with 6-min walking test (p = 0.002) and peak oxygen consumption (p = 0.001). Conclusions: NP concentration is increased in patients with PAH and inoperable CTEPH. Elevated NP concentration is associated with adverse clinical outcomes and correlates with clinical parameters.

Right heart catheterization procedures in patients with suspicion of pulmonary hypertension – experiences of a tertiary center

Introduction Right heart catheterization (RHC) is an invasive procedure providing direct and accurate measurements of hemodynamics of the cardiovascular system. Acute pulmonary vasoreactivity testing (APVT) following basal RHC in some patients is an established tool evaluating the reversibility of hypertension in the pulmonary vasculature. Aim We sought to assess the most common indications, vascular approaches and complications during RHC in a single high-volume center. Material and methods A total of 534 RHC procedures in 348 patients (64% male) were performed. The prospective registry was carried out for 28 months. Collected data included indications for RHC, vascular approaches, hemodynamic and clinical data, complications and response of pulmonary vessels in APVT. Results In 401 (75%) procedures pulmonary hypertension (mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg) was confirmed. Left heart failure was the most common indication (55.8%), mainly ischemic (26%) or dilated cardiomyopathy (19.9%). Other indications included a suspicion of arterial (21.7%), or chronic thromboembolic pulmonary hypertension (14.6%). The right internal jugular vein approach was used in 89.1% of procedures. Acute pulmonary vasoreactivity testing was performed in 143 patients, and it was positive in 67 (46.9%) cases. Complications occurred in 21 (3.9%) procedures and included pulmonary edema (0.2%), pneumothorax (0.2%) and puncture of the artery followed by the insertion of a vascular sheath (0.4%), atrial arrhythmia (0.2%), superior vena cava dissection (0.2%), incidental artery puncture (1.1%) and local hematoma (2.2%). Conclusions The most frequent indication for RHC was left heart failure, and the most common approach was the right internal jugular vein. RHC is safe procedure with a low rate of major complications.

Znaczna poprawa kliniczna pacjenta leczonego sildenafilem z powodu nadciśnienia płucnego związanego z nadciśnieniem wrotnym

We report a case of the 59-year-old men with portal hypertension and liver cirrhosis, who developed pulmonary arterial hypertension. The first line treatment with sildenafil 20 mg 3 times daily was introduced as part of a Polish National Treatment Program. At a 6-month follow-up we noticed a significant clinical improvement: the patient’s exercise capacity and echocardiographic parameters were substantially better. In addition, the B-type natriuretic propeptide significantly decreased.

Unresectable cardiac pseudoneoplasms causing ventricular tachycardia.

Cardiac pseudoneoplasms are rare and benign. According to World Health Organization, they are classified as tumor-like lesions. We report two patients with recurrent ventricular tachycardia (VT) in whom magnetic resonance imaging revealed a pathological mass occupying a large part of the left ventricle. The localization of both tumors precluded the possibility of resection; thus, only surgical biopsy was performed. After deducting the prospect of malignancy of the tumors, we treated both patients with amiodarone and implantation of a cardioverter-defibrillator [implanted cardioverter/defibrillator (ICD)]. VT is one of many probable symptoms indicating a tumor within the heart; therefore, treatment with an ICD should only be considered after a more thorough diagnosis.