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A 85–year–old female was transferred to our ICCU for acute heart failure from one of our spoke hospitals. The patient had a long history of chronic myelofibrosis, treated with fedratinib as a second–line agent after ruxolitinib failure. During a recent hospital admission, transthoracic echocardiography already showed signs of severe pulmonary hypertension (PH) with right ventricular (RV) dysfunction, so diuretic was started. On admission the patient presented showed consistent with acute on chronic kidney injury (serum Cr 3.2 mg/dl), stable anemia (Hb 9.2 mg/dl) and markedly elevated Nt–proBNP. A pulmonary CT–scan excluded acute pulmonary embolism and lung parenchymal alterations, with a clear dilation of main pulmonary artery. Transthoracic echocardiography (figure 1) confirmed precapillary PH phenotype diagnosis. Electrocardiogram (figure 2) showed sinus rhythm; incomplete RBBB, T wave inversion in V2 to V4. The patient was initially treated with high dose furosemide, low flow oxygen; but later she needed multiple cycles of inotropes, vasopressors and combined diuretic therapy (metolazone and acetazolamide) to stabilize the clinical setting. The patient also experienced episodes of paroxysmal high ventricular rate atrial flutter. After resolution of volume overload, a right heart catheterization was performed and findings were consistent with severe precapillary pulmonary hypertension: PA pressure 90/58/40 mmHg, normal PCWP, high PVR, mild cardiac index reduction. Due to critical clinical situation, Sildenafil 20 mg was started as an off–label therapy for PH (group 5). Subsequently, the patient experienced gradual but constant hemodynamic and clinical improvement and was discharged in NYHA class II. At 1 month control, patient was stable, echocardiogram revealed improvement in right ventricle dimension, systolic function and right atrial enlargement. At three months, patient is still in an intermediate risk class for PH, so we are evaluating a combination therapy (either endothelin receptor antagonist or prostanoids), taking into account anemia and thrombocytopenia. Take home message: PH can be a complication of hematological disorders as myelofibrosis, included in “mixed” forms (group 5) of PH. In our patient, after a challenging management for acute right heart failure, an off–label PDE–5 inhibitor stabilized the clinical scenario: more data are needed on PH drugs use in this setting where up to now therapeutic strategies are lacking.

High percentage of right ventricular pacing could lead to pacing induced cardiomyopathy in a non–negligible proportion of patients. Cardiac resynchronization therapy upgrading could represent an option to improve ventricular function, reducing heart failure hospitalizations. A 82 yo male was admitted to hospital with symptomatic heart failure. He had history of complete AV block treated with dual chamber pacemaker implantation five years earlier following coronary artery bypass graft surgery, bioprosthetic aortic valve replacement and septal myectomy. He had also history of cardiac arrest occurred in the context of acute coronary syndrome treated with PCI so that ICD implant was not considered at that time, and two more HF hospitalizations in previous years. Progressive LVEF decline was documented since PM impantation until last echo showed severe left ventricular dysfunction (EF 20% from baseline EF 60%). The ECG showed sinus rhythm and wide paced QRS (206 ms) with 100% pacing percentage. A CRT device upgrading was planned. Baseline venography initially showed pervious axillary–subclavian left axis, but unfortunately the guidewire encountered a complete occlusion before entering the superior vena cava. Nevertheless, dye contrast injection documented the presence of a proximal collateral branch (fig A) having a vertical course in the thorax and reaching the lateral wall of the left ventricle with an extrapericardic course then entering the right heart through inferior vena cava. Using a IM 5Fr sheath (usually used by interventional cardiologists to cannulate internal mammary artery) we could reach this branch, inserting a guidewire and finally advancing a quadripolar lead (Fig B–C) which reached the lateral left ventricular wall. LV capture was confirmed when pacing from the three proximal poles, while the distal one caused phrenic nerve capture. The quadripolar lead was connected to the LV port of a three chamber PM along with previous implanted leads thus obtaining biventricular pacing. QRS paced was 50 ms shorter than baseline. No peri–procedural complications occurred. Lead–related venous occlusion might represent an obstacle to CRT upgrade requiring high technical expertise. The presence of a complete venous occlusion might cause procedural failure, particularly in hospitals without surgical facility. In this case, the presence of a collateral branch reaching the lateral wall of the left ventricle allowed to successfully complete the CRT upgrade.