Radosław Pietura

Recurrent Hemoptysis: An Emerging Life-Threatening Complication in Idiopathic Pulmonary Arterial Hypertension

Hemoptysis is a rarely reported complication of idiopathic pulmonary arterial hypertension (IPAH). We present the case of a 27-year-old woman with IPAH, who suffered from recurrent hemoptysis and who was treated with sitaxsentan and treprostinil and remained stable in World Health Organization functional class II. During several episodes of active hemoptysis, the patient underwent bronchial artery embolization (BAE), always with good immediate results. She developed severe respiratory insufficiency and died of electromechanical dissociation 2 days after another episode, despite effective bleeding control. Recurrent hemoptysis in patients with IPAH emerges as a potential indication for urgent placement on the lung transplant list, independent from the classic prognostic factors of functional class and indices of right-sided ventricular function. Repeated BAE should not be considered as a definitive treatment in patients with pulmonary arterial hypertension with recurrent bleeding, although it may help in bridging patients to lung transplant.

Pacemaker lead extraction and recapture of venous access: technical problems arising from extensive venous obstruction.

We report the case of the extraction of 18 year-old leads in a patient with a DDD pacemaker, and chronic obstruction of the left subclavian and innominate veins coexisting with extensive stenoses in the upper caval vein. After removal of pacing leads, angiographic guidewires were introduced via the Byrd dilatators and new pacing leads introduced with the use of long sheaths originally dedicated for transvenous left ventricular leads implantation. With this case, we discuss the problems arising during reimplantation of pacing leads in patients with chronic venous occlusion.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially life-threatening disease of the pulmonary circulation [1]. The pathogenesis of CTEPH is not entirely clear. The most accepted scenario is that of aborted recanalisation of pulmonary arteries after a thromboembolic episode. While some post-embolic residua may persist in up to 50% of survivors of acute pulmonary embolism (aPE), only 0.5% to 2% will progress to CTEPH [2, 3]. This is believed to occur in the presence of significant redistribution of flow to remaining unoccluded pulmonary bed with resulting elevation of intravascular pressure and shear stress. Remodelling of initially patent pulmonary arterioles leads to an increase in pulmonary vascular resistance similar to that observed in left-to-right shunting in congenital heart disease. Progressive uncoupling of pulmonary and right ventricular elastance results in a fall of pulmonary flow, left ventricular preload, systemic blood pressure, and right ventricular (RV) coronary perfusion leading to right heart failure with severe functional disability and eventually to death. Management of CTEPH requires precise differential diagnosis and qualification for surgical treatment by an experienced multidisciplinary team. Indeed, in operable patients pulmonary endarterectomy (PEA) is highly effective in restoring functional status and improving life expectancy. A surgical technique has been optimised and implemented worldwide by a group from San Diego – University of California [4]. Nevertheless, PEA performed in deep hypothermia and intermittent total cardiac arrest remains one of the most demanding cardiovascular interventions and is performed only in a limited number of highly dedicated centres. As an example, Papworth Hospital is the only centre performing PEA in the UK, while Marie-Lannelongue Hospital in Paris remains a referral centre for France for this type of surgery. Usually, individual cardiac surgeons are responsible for PEA in their centres, as the learning curve for this intervention has been well documented [5]. With growing experience of clinicians, radiologists, surgeons, and anaesthesiologists, an increasing proportion of patients with CTEPH may benefit from PEA despite distal, less accessible intravascular residua and/or advanced age and comorbidities. This is of paramount importance since the outcome of non-operated patients is drastically worse (Figure 1), despite identical baseline haemodynamic characteristicsand significant perioperative mortality of 2–10% in patients submitted to PEA [6]. Nevertheless, even in the leading CTEPH referral centres almost 50% patients remain on medical treatment alone, with grim perspectives regarding life quality and expectancy. Based on a large randomised trial and promising long-term effects on exercise tolerance [7, 8] direct guanylyl cyclase stimulator (riociguat) has been approved for treatment of inoperable CTEPH. Riociguat may protect patent pulmonary arterioles from progressive remodelling [9] but is unlikely to affect the culprit post-embolic residua. Recently, balloon pulmonary angioplasty (BPA) has emerged as a promising new interventional option in non-operable CTEPH. In 2001, Feinstein et al. from Harvard Medical School described a group of 18 CTEPH patients treated with BPA [10]. Of these, 16 were excluded from PEA due to distal lesions, and two due to the presence of comorbidities increasing the risk associated with surgical treatment. In total, 47 procedures were performed, thus dilating or restoring the patency of 107 arteries. In the periprocedural period, 1 patient died of reperfusion pulmonary oedema and right ventricular failure. In total, reperfusion oedema occurred in 23% of cases, and its presence correlated with the value of pulmonary artery pressure (PAP) prior to the procedure. Long-term follow-up (mean, 34 months) showed an increase in physical capacity, manifesting itself as an improvement in NYHA class from a mean value of 3.3 prior to the procedure to a mean value of 1.8 following the procedure. Figure 1 Survival curves for patients with CTEPH treated with PEA (shadow line) and who were treated by pharmacotherapy only (solid line) – reprinted from Wieteska et al. [6] The succeeding years saw the development of the BPA technique mainly in Japanese centres. Japanese researchers have refined the BPA technique by reducing the number of segments treated during one session [11], by using smaller balloons [12], and by wider use of intravascular imaging [13, 14]. The experience of the Japanese centres shows that a series of BPA procedures performed in experienced centres has lead to regression of right ventricular dysfunction [15, 16] and are associated with an annual mortality below 5% – also in elderly patients [17]. However, it should be stressed that the population of Japanese patients with CTEPH differs from that observed in the European-American registry [18]. In a registry comprising 519 Japanese patients with CTEPH, lower median pretreatment mPAP (38 mm Hg vs. 47 mm Hg), more frequent use of PAH-like therapy (52% vs. 38%), and a significantly lower rate of cardiac surgical treatment used (14% vs. 57%) was observed [19]. The only larger group of patients in Europe who underwent BPA is that described by Andreassen et al. [20]. The Norwegian team treated 20 CTEPH patients, including 16 with distal lesions, 3 with proximal lesions who had refused PEA, and 1 patient with persistent pulmonary hypertension following surgical treatment. Prior to treatment, 85% of the patients presented with NYHA class III and IV symptoms. During recruitment to a percutaneous treatment program in the same centre, 50 PEAs were performed. In total, 73 BPA procedures were carried out (mean, 3.7 BPAs/patient; range: 2–9), thus performing angioplasty of 371 vessels – 118 segmental arteries and 253 subsegmental arteries. In the periprocedural period, two patients died, and treatment-requiring reperfusion oedema occurred in 7 cases. During 3-month follow-up, an improvement in the functional class (75% of patients in NYHA class I–II), in VO2max in cardiopulmonary exercise test (13.6 ±5.6 vs. 17.0 ±6.5, p < 0.001), in mean pulmonary artery pressure (45 ±11 mm Hg vs. 33 ±10 mm Hg, p < 0.001), in pulmonary vascular resistance (8.8 ±4.0 Wood Units vs. 5.9 ±3.6 Wood Units, p < 0.001), and in NT-pro-BNP levels (194 ±182 ng/ml vs. 90 ±119 ng/ml, p = 0.007) was achieved. Follow-up angiography revealed no restenosis. In Poland, the first BPA procedure was performed in 2013 [21]. Until now, the experience of our team includes 37 BPA procedures, which consisted of angioplasty of 105 vessels in 20 patients with CTEPH. Seventeen patients were excluded from surgical treatment by an experienced PEA cardiac surgery team, and in 3 patients persistent pulmonary hypertension persisted after PEA. Eighty-two percent of patients received PAH-like therapy – most frequently sildenafil. In the periprocedural period, two patients died of severe reperfusion oedema and severe hypoxaemia unresponsive to oxygen therapy (including mechanical ventilation). Those 2 patients were disqualified from PEA due to the presence of extensive lung cavities related to previous mycobacterial infection and due to significant comorbidities and advanced age, respectively – but not because of distal localisation of thrombi. All patients who underwent BPA because of distal lesion localisation survived. In technical terms, BPA does not significantly differ from balloon angioplasty performed in other vessels (Figure 2). Nevertheless, the complicated anatomy of the pulmonary tree, the necessity to advance the instruments through enlarged right heart chambers, and the fact that pulmonary vessels can be easily damaged with the guide wire or balloon catheter requires specific experience. It is not recommended that BPA procedures be performed by cardiologists or interventional radiologists who have experience in other vascular regions but no experience in interventions within the pulmonary circulation. During one procedure, no more than two segmental arteries or their subsegmental equivalents should be dilated due to the risk of reperfusion oedema. Reperfusion oedema results from redistribution of blood flow to areas supplied by dilated vessels, in which vascular resistance has abruptly decreased. This may cause blood cells to migrate into the alveoli, excluding them from gas exchange. One way to prevent reperfusion oedema is to undersize the balloon catheter being used, on the basis of angiography, or by means of intravascular ultrasound (IVUS) or optical coherence tomography (OCT). Also, pressure distal to a residual lesion and a gradient across the lesion can be measured by means of an fractional flow reserve (FFR) probe [22]. As there is no tendency towards restenosis, it is unnecessary to use stents. The results achieved in the group of patients who have completed a series of BPA procedures are very encouraging. A reduction in mean PAP from baseline 58 ±6 mm Hg to 41 ±9 mm Hg and in PVR from 11.7 ±4.3 Wood units to 6.6 ±2.2 Wood units was achieved in our series. The haemodynamic improvement corresponds with an improvement in exercise tolerance. Prior to BPA procedures, 95% of patients were in NYHA class III and IV, and the rate of patients in class III and IV decreased to 35% after treatment. A significant issue limiting growth in the number of such procedures performed in Poland is that BPA is not reimbursed by the National Health Fund. Figure 2 Balloon pulmonary angioplasty in a 67-year-old patient with persistent form of CTEPH. Left panel (A) presents the angiogram of occluded segmental pulmonary artery of left lower lobe. The BPA results in reperfusion of the vessel – right panel ( ...

Improvement in Quality of Life and Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty

BACKGROUND The effect of balloon pulmonary angioplasty (BPA) on improvement in functional and hemodynamic parameters in chronic thromboembolic pulmonary hypertension (CTEPH) is known, but the quality of life (QoL) of patients treated with BPA has never been studied before.Methods and Results:Twenty-five patients with inoperable or persistent CTEPH were enrolled in the study and filled out the 36-item Short Form (SF-36v2) questionnaire twice: prior to commencement of BPA treatment and after ≥3 BPA sessions. In addition WHO functional class, distance on the 6-min walk test (6MWT) and hemodynamic parameters such as right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), cardiac index (CI) and pulmonary vascular resistance (PVR) were assessed. QoL improved significantly in all domains, except for physical pain. Improvement in RAP (10.5±3.4 vs. 6.2±2.2 mmHg; P<0.05), mPAP (51.7±10.6 vs. 35.0±9.1 mmHg; P<0.05), CI (2.2±0.5 vs. 2.5±0.4 L/min·m2; P=0.04), PVR (10.4±3.9 vs. 5.5±2.2 Wood units; P<0.05), functional class (96% vs. 20% in WHO class III and IV, P<0.05) and improvement in 6MWT distance (323±135 vs. 410±109 m; P<0.05) was observed. The only significant correlation was between the mental component summary score of QoL after completion of treatment and percentage improvement in the 6MWT (-0.404, P<0.05). CONCLUSIONS Alongside improvement in functional and hemodynamic parameters, BPA also provides significant improvement in QoL.

Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension.

A 43-year-old female had been diagnosed as having chronic thromboembolic pulmonary hypertension (CTEPH) two years previously with pulmonary artery (PA) thrombus detected by computed tomography (CT) (Fig. 1) and precapillary pulmonary hypertension (mean PA pressure [mPAP] 56 mm Hg, cardiac ouput [CO] 6.03 L/min, PA wedge pressure 8 mm Hg, pulmonary vascular resistance [PVR] 7.96 Wood units) by right heart catheterisation (RHC). She was referred to the cardiac surgeon and was not qualified for pulmonary endarterectomy. The patient was treated with sildenafil (off-label) for one year with no improvement. She was admitted to our institution in July 2013 presenting symptoms of class III NYHA heart failure. Echocardiography revealed the progression of right ventricular failure (Fig. 2). Selective pulmonary arteriography confirmed distal localisation of thrombi in segmental arteries of left lower lobe (Fig. 3). Balloon pulmonary angioplasty (BPA) of two segmental arteries was performed, resulting in an improvement in control angiography (Fig. 4). The next BPA of another two segmental arteries was performed after three months. There were no complications during or after the procedures. Subsequent RHC confirmed an improvement (mPAP 33 mm Hg , CO 4.88 L/min, PVR 4.50 Wood units). The patient presented clinical recovery and symptoms of class II NYHA. Pulmonary endarterectomy is the only potentially curative treatment for CTEPH. The prognosis of CTEPH has been reported to be poor when mPAP is > 30 mm Hg. BPA seems to be a promising strategy for inoperable CTEPH. Reperfusion pulmonary injury is the major complication after BPA. To reduce the size of the area of this complication, it is recommended not to dilate > two vessels at the initial BPA and to perform it in a staged fashion over several separate procedures.

Endovascular embolization of varicoceles using n-butyl cyanoacrylate (NBCA) glue.

Summary Background: Varicoceles are abnormally dilated veins within the pampiniform plexus. They are caused by reflux of blood in the internal spermatic vein. The incidence of varicoceles is approximately 10–15% of the adolescent male population. The etiology of varicoceles is probably multifactorial. The diagnosis is based on Doppler US. Treatment could be endovascular or surgical. The aim of the study was to describe and evaluate a novel method of endovascular embolization of varicoceles using n-butyl cyanoacrylate (NBCA) glue. Material/Methods: 17 patients were subjected to endovascular treatment of varicoceles using NBCA. A 2.8 Fr microcatheter and a 1:1 mixture of NBCA and lipiodol were used for embolization of the spermatic vein. Results: All 17 procedures were successful. There were no complications. Discussion: Embolization of varicoceles using NBCA glue is efficient and safe for all patients. The method should be considered as a method of choice in all patients. Phlebography and Valsalva maneuver are crucial for technical success and avoidance of complications. Conclusions: Endovascular treatment of varicoceles using NBCA glue is very effective and safe.

Transvenous extraction of an eight-year-old ventricular lead accidentally implanted into the left ventricle

Incorrect implantation of a ventricular pacemaker (PM) lead into the left ventricle (LV) is a known problem associated with permanent pacing. The optimal management of such cases identified late has not been clearly established. Generally acceptable management options are: open-chest cardiac surgery using cardio-pulmonary bypass, chronic anticoagulation and antiplatelet-drugs therapy. Rarely, the problem is solved by percutaneous LV lead extraction. We present a case of a patient with DDD pacing and ventricular lead implanted incorrectly into the LV apex region via an atrial septal defect eight years ago. Chronic PM pocket infection developed after replacement of the device. Both leads were extracted percutaneously, and the embolic protection system (Filter-Wire EZ, Boston Scientific) was used to reduce cerebral circulation embolism. The hardest connective tissue adhesions affecting the lead and the anodal ring were found in the LV. Less dense surrounding fibrous tissue around the lead was present at all levels of the venous course of the lead and in the right atrium. Very small fragments of apparently connective tissue remnants were found in cerebral circulation protection filters, and had been removed after the procedure. We conclude that old, permanently implanted LV leads may be extracted percutaneously, especially when there is an increased risk of cardiac surgery, or where the patients consent for surgical treatment is lacking. In order to perform the procedure it is recommended to establish a cerebral protection system and intraoperative transoesophageal echocardiography which are mandatory for successful lead removal.

Broken leads with proximal endings in the cardiovascular system: Serious consequences and extraction diffi culties

BACKGROUND Retrospective analysis of effectiveness, technical problems, and complications of transvenous extraction of leads with the free endings migrated to the cardiovascular system (CVS). METHODS A 5-year-old database of transvenous lead extraction (TLE) procedures comprising 906 patients with 1563 leads being removed was analyzed. TLE procedures of leads migrated in the CVS were compared with TLE procedures of leads with their proximal ends accessible in the pacemaker/implantable cardioverter-defibrillator (PM/ICD) pocket. RESULTS In our material, the phenomenon of leads migration occurred in 5% of patients referred for TLE and affected most frequently unipolar and atrial leads. The presence of migrating leads was associated with local venous occlusion in 64% of patients. Removal of migrating leads required other techniques than extraction of leads with their proximal ends accessible in the PM/ICD pocket. More than 95% of migrating leads were extracted transvenously, but procedures were significantly longer. The presence of other leads made extraction of migrated leads even more complicated. Effectiveness and complication rates for removal of migrated leads and leads accessible in the PM/ICD pocket were similar. CONCLUSIONS We postulate that every lead migrating in the CVS should be considered for TLE. However, this extraction is technically more difficult and challenging than extraction of leads accessible in the PM/ICD pocket.

Effectiveness, safety, and long-term outcomes of non-powered mechanical sheaths for transvenous lead extraction

Aims To analyse the effectiveness, safety and long-term outcomes of conventional non-powered mechanical systems for transvenous lead extraction (TLE) performed by experienced first operators. Outcomes were assessed according to lead location and type of operating room in which the procedure was performed. Methods and results Data from 2049 patients (mean age: 65 years), with infectious (40%) or non-infectious (60%) indications, were analysed over a mean of 3.37 (±2.29) years. A total of 3426 leads were extracted; and, overall, 95% full procedural, 4% partial procedural, and 98% clinical success were demonstrated. Within the patient cohort, 1.8% (37/2049) experienced major complications, with cardiac tamponade being predominant (30/37). Cardiac tamponade was identified as the main cause of mortality, as well as the cause of all procedure-related deaths (6/2049; 0.3%). Cardiac tamponade occurred in 1.8% of atrial and 0.3% of right ventricular lead extractions, with fatal tamponade reported in 9% of atrial, 40% of ventricular, and 67% of coronary sinus lead extractions. No association between lead location and cardiac tamponade-related mortality was observed; however, lead location did affect the success of pericardiocentesis. The cardiac tamponade-related mortality rate was 37% when TLE was performed in an electrophysiology laboratory. No deaths were reported when the procedure was performed in a cardiac surgery or hybrid operating room. Long-term survival was improved when TLE was performed due to non-infectious indications, rather than pocket infection or lead-related endocarditis (P < 0.001). Conclusion Using conventional non-powered mechanical sheaths, TLE was effective even in patients at high risk of complications.

Complementary role of magnetic resonance imaging after ultrasound examination in assessing fetal renal agenesis: a case report.

IntroductionUltrasonography is used routinely during pregnancy to screen and detect fetal abnormalities. However, there are some conditions like anhydramnios (a prevalent state in renal agenesis) or maternal obesity that may limit the diagnostic accuracy of ultrasonography. Magnetic resonance imaging has proven to be useful when ultrasound alone is insufficient to make a correct diagnosis.Case presentationWe present the case of a 22-year-old Caucasian woman who was admitted to our unit at the 26th week of gestation for a detailed anatomy scan. Anhydramnios and failure to visualize the kidneys, bladder and renal vessels were confirmed with the use of sonography in our department. Since the lack of amniotic fluid limited the acoustic window for fetal ultrasonography, a magnetic resonance imaging scan was requested to confirm suspected renal agenesis. A fetal magnetic resonance imaging scan was performed and confirmed the suspected diagnosis. A baby boy was born by breech vaginal delivery after spontaneous onset of labor at the 34th week of gestation. The boy weighed 1690g, with Apgar scores of 6 and 4 at two and five minutes respectively, and died one hour after delivery. The diagnosis of bilateral renal agenesis was confirmed on autopsy.ConclusionsThe aim of this study was to evaluate the potential contribution of magnetic resonance imaging in diagnostic procedure after inconclusive ultrasound examination during the assessment of fetal urinary tract abnormalities in the third trimester.