Postcardiac injury syndrome caused by radiofrequency catheter ablation of persistent atrial fibrillation: severe pulmonary arterial hypertension with severe tricuspid regurgitation: a rare case report and literature review.

BACKGROUND: Postcardiac injury syndrome (PCIS) is an easy-to-miss diagnosis, but it is not an uncommon complication. The phenomenon of echocardiography (ECHO) showing both severe pulmonary arterial hypertension (PAH) and severe tricuspid regurgitation (TR) is indeed rare in PCIS after extensive radiofrequency ablation. CASE PRESENTATION: A 70-year-old male was diagnosed with persistent atrial fibrillation. The patient received radiofrequency catheter ablation due to his atrial fibrillation being refractory to antiarrhythmic drugs. After the anatomical three-dimensional models were created, ablations were performed on the left and right pulmonary veins, roof linear and bottom linear of the left atrium, and the cavo-tricuspid isthmus. The patient was discharged in sinus rhythm (SR). After 3 days, he was admitted to the hospital for gradually worsening dyspnea. Laboratory examination showed a normal leukocyte count with an increased percentage of neutrophils. The erythrocyte sedimentation rate, C-reactive protein concentration, interleukin-6, and N-terminal pro-B-type natriuretic peptide were elevated. ECG exhibited SR, V1-V4 of precordial lead P-wave amplitude which was increased but not prolonged, PR segment depression, and ST-segment elevation. Computed tomography angiography of the pulmonary artery revealed that the lung had scattered high-density flocculent flakes and a small amount of pleural and pericardial effusion. Local pericardial thickening was seen. ECHO showed severe PAH with severe TR. Diuretics and vasodilators did not relieve the symptoms. Tumors, tuberculosis, and immune system diseases were all excluded. Considering the patient's diagnosis of PCIS, the patient was treated with steroids. The patient recovered on the 19th day post ablation. The patient's condition was maintained until 2 years of follow-up. CONCLUSIONS: The phenomenon of ECHO showing severe PAH with severe TR is indeed rare in PCIS. Due to the lack of diagnostic criteria, such patients are easily misdiagnosed, leading to a poor prognosis.

Single-Center Experience of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension: The Results from the Tabriz University Medical Sciences (TUMS) CTEPH Program.

INTRODUCTION: Pulmonary endarterectomy (PEA) remains the preferred and potentially curative option for patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to report the results of PEA for CTEPH in a tertiary center in Tabriz, Iran. METHODS: We analyzed the results of 42 CTEPH patients undergoing PEA, who were enrolled in the Tabriz University of Medical Sciences (TUMS-CTEPH) from January 2016 to October 2020. The main outcome measures included the New York Heart Association (NYHA) functional classification, the 6-Minute Walk Distance, hemodynamic measures in right heart catheterization, morbidity, and mortality. RESULTS: There was a significant improvement in the NYHA function class (2.6 ± 0.5 vs 1.1 ± 0.34), mean pulmonary arterial pressure (47.1 ± 13 vs 27.9 ± 8 mm Hg), cardiac output (4.3 ± 1.06 vs 5.9 ± 1.2 L/min), and pulmonary vascular resistance (709.4 ± 297.5 vs 214 ± 77 dyn s/cm5). Fifteen patients (35%) developed complications. The most common complication (10 [23%]) was reperfusion injury. Also, postsurgical mortality was 4% during hospital admission and 1-year follow-up. CONCLUSION: This is the first single-center report of PEA from Iran. Post-PEA and 1-year survival were acceptable as a referral center. PEA can be performed safe with low mortality. Greater awareness of PEA and patients' access to experienced CTEPH centers are important issues.

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement.

BACKGROUND: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death. METHODS: A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and non-obstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a framework of relevant clinical questions, we review the available evidence guiding operative risk, risk assessment, preoperative optimisation, and perioperative management, and identifying areas for future research. RESULTS: Reported 30 day mortality after non-cardiac and non-obstetric surgery ranges between 2% and 18% in patients with PH undergoing elective procedures, and increases to 15-50% for emergency surgery, with complications and death usually relating to acute right ventricular failure. Risk factors for mortality include procedure-specific and patient-related factors, especially markers of PH severity (e.g. pulmonary haemodynamics, poor exercise performance, and right ventricular dysfunction). Most studies highlight the importance of individualised preoperative risk assessment and optimisation and advanced perioperative planning. CONCLUSIONS: With an increasing number of patients requiring surgery in specialist and non-specialist PH centres, a systematic, evidence-based, multidisciplinary approach is required to minimise complications. Adequate risk stratification and a tailored-individualised perioperative plan is paramount.

A rare childhood case of Behcet's disease and chronic thromboembolic pulmonary hypertension.

BACKGROUND: Pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH) are rare complications of Behcet's disease, especially in pediatric patients. AIMS/METHODS/RESULTS/CONCLUSIONS: This case report highlights a presentation of CTEPH in an adolescent with Behcet's disease. A multidisciplinary approach was required for managing this patient's CTEPH, which successfully reversed the patient's pulmonary hypertension.

Outcomes of infants and children undergoing surgical repair of ventricular septal defect: a review of the literature and implications for research with an emphasis on pulmonary artery hypertension.

BACKGROUND: Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect. METHODS: We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes. RESULTS: Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair. CONCLUSION: Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.

Surgical treatment of chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease characterized by a mean pulmonary artery pressure that exceeds 25 mm Hg and is caused by intraluminal thrombi organisation, stenosis and occlusions of pulmonary artery and its branches and peripheral vascular remodelation. It is a chronic complication of acute pulmonary embolism. The obstruction of pulmonary artery branches increases pulmonary vascular resistance (PVR) and this leads to the right ventricular overload and right-sided heart failure. The treatment of choice is surgical pulmonary endarterectomy (PEA), a procedure that is performed in deep hypothermic cardiac arrest. The only center that specializes into the surgical treatment of patients with CTEPH in the Czech Republic is the Complex Cardiovascular Centre at the General Teaching Hospital in Prague. Between years 2004-2017 there were 314 patients opera-ted (including 50 patients from Slovakia, where this treatment is not available). Patients with peripheral type of CTEPH, who are not indicated for operation and also patients with residual pulmonary hypertension after PEA can be indicated for specific vasodilatation therapy. In indicated cases the treatment may involve the balloon angioplasty or lung transplantation.

[Radiofrequency Pulmonary Artery Ablation for Treatment of Residual Pulmonary Hypertension After Pulmonary Endarterectomy].

OBJECTIVE: to assess the safety and efficiency of radiofrequency pulmonary artery ablation for treatment of residual pulmonary hypertension after pulmonary endarterectomy. MATERIAL AND METHODS: Radiofrequency pulmonary artery denervation (PADN) was performed in 16 patients (10 men, mean age 39 years [26; 51]). Indication for PADN was mean pulmonary artery pressure (PAP) >25 mm Hg with absence of proximal pulmonary artery lesion according to computer tomography. PADN procedure was performed with nonfluoroscopic 3D navigation system. RESULTS: After PADN mean PAP decreased from 37.3 mm Hg [29; 38] to 24.6 mm Hg [17; 30] (p=0.01); pulmonary vascular resistance decreased from 672 dyn·s·c -5 [387; 566] to 386 dyn·s·c -5 [155; 449] (р=0.02); cardiac output increased from 3.4 l / min [3.2; 3.4] to 3.5 l / min [3.2; 4.0] (p=0.4); 6­minute walk test distance increased from 427 meters [352; 510] to 447 meters [370; 525] (p=0.16), respectively. CONCLUSION: Initial results allow to assume that radiofrequency pulmonary artery denervation combined with optimal medical therapy may take its rightful place in the treatment of this category of patients.

Radiofrequency Pulmonary Artery Ablation for Treatment of Residual Pulmonary Hypertension After Pulmonary Endarterectomy.

OBJECTIVE: to assess the safety and efficiency of radiofrequency pulmonary artery ablation for treatment of residual pulmonary hypertension after pulmonary endarterectomy. MATERIAL AND METHODS: Radiofrequency pulmonary artery denervation (PADN) was performed in 16 patients (10 men, mean age 39 years [26; 51]). Indication for PADN was mean pulmonary artery pressure (PAP) >25 mm Hg with absence of proximal pulmonary artery lesion according to computer tomography. PADN procedure was performed with nonfluoroscopic 3D navigation system. RESULTS: After PADN mean PAP decreased from 37.3 mm Hg [29; 38] to 24.6 mm Hg [17; 30] (p=0.01); pulmonary vascular resistance decreased from 672 dyn∙s∙c-5 [387; 566] to 386 dyn∙s∙c-5 [155; 449] (р=0.02); cardiac output increased from 3.4 l/min [3.2; 3.4] to 3.5 l/ min [3.2; 4.0] (p=0.4); 6-minute walk test distance increased from 427 meters [352; 510] to 447 meters [370; 525] (p=0.16), respectively. CONCLUSION: Initial results allow to assume that radiofrequency pulmonary artery denervation combined with optimal medical therapy may take its rightful place in the treatment of this category of patients.

Experiences of adolescent lung transplant recipients: A qualitative study.

Many young transplant recipients experience psychological distress and adjustment difficulties, yet there is little research investigating lung transplantation from the recipients' perspective. This qualitative study aimed to explore experiences of young people who underwent lung transplantation. Semi-structured interviews were conducted with six lung transplant recipients (aged 15-18). Interviews were analysed using IPA, a qualitative research approach examining how people make sense of their major life experiences. The analysis revealed three master themes: "Living with Dodgy Lungs" outlined how participants dealt with their experiences, managing through accepting or discussing their feelings with others, although talking was often difficult. "The Big Deal" reflected participants' experiences of the process, their expectations, and the contrast of their lives pre- and post-transplant. Inherent in their accounts was the profound meaning ascribed to transplantation, the emotional turmoil, and impact on their lives. "A Sense of Self" illustrated participants' developing identities within their social contexts and at times isolating experiences. The results highlight key areas where adolescent lung transplant recipients could be supported by clinicians, enabling the promotion of psychological well-being. Examples include supporting identity integration post-transplant, facilitating social inclusion, considering alternative means of support, and involving adolescents in healthcare decisions.

The effective systematic heparin pre-treatment on thrombus formation on pulmonary artery catheter tips during pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a randomized, double-blind study.

Pulmonary artery (PA) catheters are routinely used for hemodynamic management in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA). Tip-associated thrombi are frequently detected and might increase the peri-operative risk in these patients. The aim of the study was to investigate the effects of low-dose heparinization before the insertion of the PA catheter on thrombus formation and thrombus weight during PEA surgery. From September 2013 to February 2015, 60 CTEPH patients undergoing PEA were included in the study and randomized into two groups of 30 patients each, including a heparin group (heparin bolus (70 IU per kg body weight) administration before PA catheter insertion) and a control group (pretreatment with placebo). During the PEA procedure the distal part of the PA catheter was drawn out of the PA and thrombus presence and weight were recorded. There were no significant differences in baseline characteristics between the two groups. Twelve patients (20%) had thrombophilic disorders. In the control group, thrombi were detected in 17 patients (57%) with a median thrombus weight of 27 mg (IQR 41). In the heparin group, tip-associated thrombi were found in five patients (17%) with a median weight of 12 mg (IQR 7). There were no bleeding complications in either group. This study demonstrates a high risk of PA catheter-related thrombi in patients with CTEPH. Prophylactic administration of low-dose heparin reduces thrombus formation and thrombus weight without an increased rate of bleeding complications.

Current status of domino heart transplantation.

Domino heart transplant, wherein the explanted heart from the recipient of an en-bloc heart-lung is utilized for a second recipient, represents a unique surgical strategy for patients with end-stage heart failure. With a better understanding of the potential advantages and disadvantages of this procedure, its selective use in the current era can improve and maximize organ allocation in the United States. In this report, we reviewed the current status of domino heart transplantation.

[Critical Care Managements after Lung Transplantation].

Lung transplantation is a viable treatment option for patients with end-stage lung diseases such as interstitial pneumonia/pulmonary fibrosis, emphysema, pulmonary hypertension, and so on. Collecting available clinical, physiological, serological, and surgical information of both donor and recipient is vital when planning relevant postoperative managements. The goal of the managements is to keep the transplanted lung (s) functional while preventing/treating infection, rejection, and ischemiareperfusion lung injury. Immunosuppressive therapy, anti-mycobacterial/viral therapy, and cardio-pulmonary supports should be optimized without causing unfavorable side-effects that can lead to kidney, liver, digestive and neurological malfunction. During the post-transplant intensive care period, satisfying the endorgan oxygen requirement is the key to maintain vital organ stability. Aggressive rehabilitation should be utilized as soon as the hemodynamic status allows it. Deep venous thrombosis and subsequent pulmonary embolism should be prevented by giving anti-coagulants and active mobilization, because the incidence could be underrecognized. Avoiding multifactorial allograft injuries can improve not only short-term graft function, but also long-term patients' outcome after lung transplantation.

[5-year surgical results in patients with chronic postembolic pulmonary hypertension]. / Pyatiletnie rezul'taty khirurgicheskogo lecheniya patsientov s khronicheskoi postembolicheskoi legochnoi gipertenziei.

AIM: To evaluate 5-year results of surgical treatment of patients with chronic postembolic pulmonary hypertension. MATERIAL AND METHODS: 170 patients with chronic postembolic pulmonary hypertension underwent pulmonary thrombendarterectomy. Mean age was 48±21.5 years. The operation was carried out using standard technique with hypothermia and circulatory arrest. We have analyzed clinical and functional status of 47 patients (23 males) prior to surgery and in long-term postoperative period (5-6 years). Distance of 6-minute walk test, mean pulmonary artery pressure and vascular resistance according to right heart catheterization, RV ejection fraction and volumes according to echocardiography, pulmonary artery diameter according to CT-angiography were analyzed. RESULTS: In remote postoperative period average distance of 6-minute walk test was increased by 2.8 times compared with initial values. Right heart catheterization revealed reduction of the average pulmonary pressure from 54.04±23.35 to 25.16±15.41 mmHg and vascular resistance from 539.66±120.59 dyn·sec·cm-5 to 101.39±89.20 dyn·sec·cm-5. Echocardiography showed increase of RV EF from 36±2.3 to 51.8±4.1% and decrease of RV end-diastolic volume from 109.4±39.2 to 39.1±6.8 ml. According CT-angiography there was decrease of pulmonary trunk diameter from 35.10±5.25 to 30.30±8.65 mm. CONCLUSION: Our 5-year data show that pulmonary thrombendarterectomy has high long-term effectiveness in patients with proximal form of chronic postembolic pulmonary hypertension.

MELD exceptions for portopulmonary hypertension: current policy and future implementation.

Since 2006, waitlist candidates with portopulmonary hypertension (POPH) have been eligible for standardized Model for End-Stage Liver Disease (MELD) exception points. However, there are no data evaluating the current POPH exception policy and its implementation. We used Organ Procurement and Transplantation Network (OPTN) data to compare outcomes of patients with approved POPH MELD exceptions from 2006 to 2012 to all nonexception waitlist candidates during this period. Since 2006, 155 waitlist candidates had approved POPH MELD exceptions, with only 73 (47.1%) meeting the formal OPTN exception criteria. Furthermore, over one-third of those with approved POPH exceptions either did not fulfill hemodynamic criteria consistent with POPH or had missing data, with 80% of such patients receiving a transplant based on receiving exception points. In multivariable multistate survival models, waitlist candidates with POPH MELD exceptions had an increased risk of death compared to nonexception waitlist candidates, regardless of whether they did (hazard ratio [HR]: 2.46, 95% confidence interval [CI]: 1.73-3.52; n = 100) or did not (HR: 1.60, 95% CI: 1.04-2.47; n = 55) have hemodynamic criteria consistent with POPH. These data highlight the need for OPTN/UNOS to reconsider not only the policy for POPH MELD exceptions, but also the process by which such points are awarded.

Causative role of infection in chronic non-thromboembolic pulmonary hypertension following ventriculo-atrial shunt.

A severe complication of ventriculo-atrial (VA) shunt placement for treatment of hydrocephalus is chronic thromboembolic pulmonary hypertension (CTEPH). We report here a patient with a VA shunt for treatment of hydrocephalus who presented two consecutive episodes of VA shunt-induced infection by Staphylococcus epidermidis and who rapidly developed chronic PH. Extensive radiological investigations and normal ventilation/perfusion lung scan allowed us to rule out CTEPH. To our knowledge, no other case of chronic pulmonary hypertension (PH) related to VA shunt insertion has been reported so far. PH in this patient with VA shunt is clinically distinct from CTEPH and has been caused by VA shunt-induced S. epidermidis infection per se.

Lung Transplantation in Patients With Systemic Scleroderma-Description of the First Consecutive Cases in Poland: Case Series Report and a Short Literature Review.

Pulmonary complications of systemic scleroderma (SSc), such as interstitial lung disease and pulmonary hypertension (PH), are responsible for up to 60% of deaths among patients. For many years, most centers considered SSc a contraindication to lung transplantation (LTx); however, recent publications show that appropriately selected SSc candidates for LTx give results comparable to patients with idiopathic PH or idiopathic pulmonary fibrosis. This paper presents the cases of a 60-year-old male patient (patient 1) and a 42-year-old female patient (patient 2) diagnosed with SSc in 2019 and 2013, respectively. In both patients, interstitial-fibrotic changes in the lungs leading to respiratory failure were confirmed by high-resolution computed tomography as well as pulmonary hypertension (WHO group 3), which was also diagnosed during right heart catheterization. In both cases, despite pharmacotherapy, pulmonary fibrosis progressed, leading to severe respiratory failure. The patients were referred for LTx qualification. LTx was possible to consider in patients due to the lack of significant changes in other internal organs. Double LTx was successfully performed in both patients (patient 1-July 19, 2022; patient 2-September 14, 2022). They were discharged from the hospital in good condition on the 22nd and 20th postoperative day, respectively. LTx is a last-chance therapy that saves lives among patients with extreme respiratory failure in the course of SSc. It prolongs and improves the quality of life. The selection of appropriate patients is key to the success of the procedure.

Resolutive pulmonary endarterectomy in a non-compliant patient with systemic lupus erythematosus and antiphospholipid syndrome.

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) have poor prognosis, and pulmonary endarterectomy (PEA) is considered the treatment of choice for this condition. We report a case and review the literature of successful PEA for CTEPH due to antiphospholipid syndrome associated with systemic lupus erythematosus. The definitive and decisive approach needed to treat this high-risk patient with a history of comorbidity, long-term illness and poor compliance was found with a therapy of PEA.

Combined heart-lung transplantation: a perspective on the past and the future.

During the last 20 years, there has been a shift away from combined heart-lung transplantation (HLT) in favor of bilateral lung transplantation. This paradigm shift allowed for the donor heart to be transplanted to another patient. However, HLT remains to be the definitive surgical treatment for certain congenital heart disorders and Eisenmenger's syndrome. With a growing population of adult patients with congenital heart disease, there remains a need for HLT. This article provides a perspective on the past and the future of HLT.

Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension: Relationship between treated branches and outcome.

BACKGROUND: In patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) it is important to minimize residual obstructions, in order to achieve low postoperative pulmonary vascular resistances and better clinical results. The aim of the study was to test the hypothesis that the greater the number of pulmonary artery branches treated at surgery, the better the hemodynamic and clinical outcome after PEA. METHODS: In 564 consecutive CTEPH patients undergoing PEA the count of the number of treated branches was performed directly on the surgical specimens. Post-operative follow-up visits were scheduled at 3 months and 12 months after surgery including right heart catheterization and modified Bruce test. RESULTS: The population was divided into tertiles based on the number of treated branches: Group 1 (from 4 to 30 treated branches, n = 194 patients); Group 2 (from 31 to 43 treated branches, n = 190 patients); Group 3 (from 44 to 100 treated branches, n = 180 patients). At 3 and at 12 months after PEA, after adjustment for confounders, patients in the highest tertile of treated branches had significantly lower values of pulmonary vascular resistance and higher values of pulmonary arterial compliance as compared to the other two groups (p < 0.002). Hospital mortality was 3% in Group 3, 6% in Group 2 and 10% in Group 1 (overall p = 0.035). CONCLUSIONS: In CTEPH patients undergoing PEA, a higher number of treated pulmonary artery branches is associated with a better hemodynamic and a better clinical outcome at 3 months and 12 months after surgery.