Self‐repositioning of an embolized patent ductus arteriosus device—a nightmare turned into sweet dreams

A 7‐month‐old boy was admitted for the device closure of symptomatic patent ductus arteriosus (PDA) with moderate pulmonary hypertension. The PDA measured 4.2 mm with adequate ampulla. It was closed with an 8–6 mm Heart R device from pulmonary artery (PA) end. Post extubation, the device embolized to proximal descending thoracic aorta just distal to PDA ampulla. While attempting to snare from the venous side, the device self‐repositioned to PDA. It was stable thereafter and patient was discharged after 2 days. We report a complication, which got self‐corrected. © 2014 Wiley Periodicals, Inc.     

Transcatheter closure of a non-restrictive patent ductus arteriosus with an Amplatzer muscular ventricular septal defect occluder

We report on successful occlusion of a large unrestrictive PDA in an adult patient with pulmonary hypertension.     

Successful closure of a large PDA with an Amplazter septal occluder in a child with heart failure and pulmonary hypertension--a case report

Transcatheter methods for closure of patent ductus arteriosus (PDA) have been known as an effective technique for many years. The devices designed for interventional closure of PDA, coexisting with elevated pulmonary artery pressure are still far from ideal and there is a significant risk of embolisation into the aorta. We present a case of a 14-year-old girl with a large PDA, pulmonary hypertension and congestive heart failure. She underwent successful PDA closure with an Amplatzer atrial septal occluder (ASO), a device originally designed to close an atrial septal defect.     

Transcatheter occlusion of gigantic persistent ductus arteriosus (PDA) using a custom‐made persistent ductus arteriosus occluder

We reported transcatheter closure of gigantic persistent ductus arteriosus (PDA) complicated by severe pulmonary hypertension (PH) using a custom‐made PDA occluder. A 19‐year‐old lady weighing 45 kg visited to our Heart Saving Project in Mongolia with a chief complaint of shortness of breath. Contrast CT scan showed ellipsoidal section of PDA whose long axis being 28 mm, and the short axis of 21 mm. A custom‐made PDA occluder, whose retention skirt, the aortic side, and the pulmonic side diameter of the body were 54, 36, 34 mm, respectively, was successfully deployed using 14‐Fr sheath. Pulmonary pressure decreased around a half compared to before closure. A custom‐made duct occluder could be a reasonable and cost‐effective choice for transcatheter closure of gigantic PDA complicated by severe PH. © 2015 Wiley Periodicals, Inc.     

Transcatheter closure of patent ductus arteriosus and aorto‐pulmonary vessels using non‐ferromagnetic Inconel MReye embolization coils

Objectives . We report the use of non‐ferromagnetic embolization coils for transcatheter PDA closure. Background . Transcatheter patent ductus arteriosus (PDA) closure has been performed for 40 years. A number of devices have been used with varying degrees of success. Gianturco embolization coils have been used frequently since 1992 with excellent results. These coils are a stainless steel alloy, and create an artifact when subsequent MRI imaging is performed. Methods . Eight patients underwent right and left heart catheterization and transcatheter PDA closure. Angiography displayed a PDA with left to right shunting. The minimum PDA diameter was measured. An Inconel MReye coil was implanted using standard retrograde technique. A postimplant angiogram was performed. Evaluations were performed the following morning and after 2 months. Results . The median age was 5.5 years, median weight was 24 kg. The PDA minimum diameter was 1.7 mm (range 1.4–2.4 mm), with a median Qp:Qs=1.33:1. In all patients, the PDA was completely immediately closed using one Inconel coil. Two patients also had a small aorto‐pulmonary collateral vessel that was occluded using a separate Inconel coil. All patients had follow‐up evaluation the following day; the PDA remained completely occluded and there was no obstruction of the pulmonary artery branches or descending aorta. Seven patients had subsequent follow‐up and echocardiograms; the PDA remained completely occluded. There were no complications. Conclusion . The Inconel MReye coil is safe and effective for coil occlusion of small PDA and aorto‐pulmonary vessels. Additional studies are needed to define the maximum vessel diameter for Inconel coil occlusion. © 2008 Wiley‐Liss, Inc.     

Emergency trans‐catheter coronary intervention for left main compression secondary to pulmonary hypertension in a 4‐year‐old child

Here, we report on the case of a 4‐year‐old child with large atrial septal defect (ASD) and severe pulmonary hypertension presenting an ischemic cardiomyopathy secondary to left main (LM) compression by a dilated pulmonary artery trunk. Despite of surgical treatment consisting in ASD closure and coronary artery bypass grafting, the patient was not weanable from ECMO. Control coronarography showed a near‐occlusion of the left mammary bypass. A rescue percutaneous LM angioplasty with drug‐eluting stent implantation was performed.     

Ductus arteriosus‐associated infective endarteritis: Lessons from the past,future perspective

Background: Since routine clinical use of antibiotics as well as surgical and catheter‐ based closure of a patent arterial duct (PDA), PDA‐associated infective endarteritis (PDA‐IE) is rare but can still occur when the ductus is still open or as it closes. Thus, clinicians should maintain a high index of concern for patients with unexplained fever.
Methods: We report on a PDA‐IE in a young infant shortly after potentially delayed obliteration of a PDA. We discuss this case report by reviewing the literature in regard to the pathogenesis (infection primary or secondary to PDA thrombus formation), clinical (new heart murmur) and diagnostic findings (transthoracic echocardiography, total body MRI, laboratory findings), and clinical outcome during mid‐term follow‐up after successful antibiotic treatment.
Results: A 7‐week‐old term infant with Staphylococcus aureus sepsis and a new heart murmur was diagnosed with PDA‐IE by transthoracic echocardiography at the pul‐ monary artery end of an obliterated PDA. Broad diagnostic workup excluded other reasons for sepsis. After 4 weeks of antibiotic treatment the vegetation reduced in size and the infant recovered completely. A review of all cases of PDA‐IE (in pediatric and adult patients) previously published was performed.
Conclusion: Nowadays, a PDA‐IE is an extremely rare, but still life‐threating condi‐ tion that may even affect patients with a nonpatent ductus arteriosus shortly after its obliteration and should be considered as infective complication in preterms, neo‐ nates, and small infants. Therefore, in septic neonates with bacteremia, transthoracic echocardiography may be integrated in the diagnostic workup, especially by fever without source and clinical signs of IE such as a new heart murmur.     

Percutaneous pulmonary valve implantation in a dysfunctional Trifecta® bioprothesis after high‐pressure balloon fracturing

A percutaneous pulmonary valve‐in‐valve (PPVIV) implantation in small surgical tissue valves may be limited due to the valve's initial diameter. Fracturing of the valve's integrity by high‐pressure balloons may enhance the diameter and facilitate subsequent PPVIV with a large valve. To the best of our knowledge, the Trifecta® valve seemed not to be accessible for fracturing. We report a case of successful 19‐mm Trifecta valve fracturing, followed by PPVIV using a 26‐mm Edwards SAPIEN 3 valve in pulmonary position. By repetitively using a high‐pressure balloon 5 mm larger than the labeled valve size, we were able to fracture the valve's integrity and implant a 26‐mm valve thereafter. Therefore, Trifecta valve appears to be suitable for valve ring fracturing and subsequent PPVIV in certain patients.     

Treatment of a degenerative stenosed CoreValve® aortic bioprosthesis by transcatheter valve‐in‐valve insertion

Background : Transcatheter aortic valve insertion (TAVI) is an emerging therapy in patients at high risk for open heart surgery. The long‐term durability of the bioprosthesis is unknown. This is the first report of a severely degeneratively stenosed 2nd generation 26 mm CoreValve^® aortic bioprosthesis which occurred five and a half years after TAVI. Methods and Results : A 92‐year‐old patient presented with decompensated heart failure NYHA class IV, pulmonary edema, and severe pulmonary hypertension. Echocardiography revealed critical AV‐stenosis due to heavily calcified bioprosthetic valve leaflets. Due to high surgical risk with an EuroSCORE of 64.97% and a STS‐mortality score of 27.0%, we decided to attempt a valve‐in‐valve insertion of a 3rd generation CoreValve^® prosthesis of the same size. Following the delicate retrograde passage of the calcified valve with a preformed stiff wire, balloon valvuloplasty of the severely stenosed CoreValve^® prosthesis under rapid right ventricular pacing was complicated by two balloon catheter ruptures. Insertion of the 3rd generation CoreValve^® prosthesis of the same size was quite complex but finally it was successfully completed. There was mild periprosthetic regurgitation and significant decrease in transaortic pressure without residual transvalvular gradient immediately after TAVI. Echocardiography and clinical follow‐up at 72 hr after TAVI confirmed excellent valve function with a decrease in systolic pulmonary artery pressure from 70 mm Hg to 35 mm Hg, increase in LV‐EF from 35% to 45%, and improvement of functional status from NYHA IV to NYHA II. The patient was discharged in good medical conditions at day eight. Conclusion : Degenerative stenosis of a CoreValve^® bioprothesis may be observed during long‐term follow‐up after successful TAVI for the treatment of severe aortic valve stenosis. A second valve‐in‐valve insertion appears feasible but may require particular interventional approaches. © 2011 Wiley Periodicals, Inc.     

Fenestrated ASD Closure in a Child with Idiopathic Pulmonary Hypertension and Exercise Desaturation

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right‐to‐left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½‐year‐old was diagnosed with pulmonary hypertension, a large atrial septal defect and right‐to‐left shunting. Medical therapy over an 18‐month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left‐to‐right atrial flow.     

A 50‐year‐old woman with haemoptysis,cough and tachypnea: cholesterol pneumonia accompanying with pulmonary artery hypertension

Lipoid pneumonia is an uncommon disease caused by the presence of lipid in the alveoli. Here we described a case of a 50‐year‐old woman with haemoptysis, cough and tachypnea, who was diagnosed with cholesterol pneumonia accompanying with pulmonary artery hypertension. The extremely high pulmonary artery pressure achieved, in this case, is alarming and should alert the physicians that the cholesterol pneumonia may be one of the underlying causes of pulmonary artery hypertension. After a treatment of methylprednisolone, her clinical symptoms were significantly improved, which suggested that steroid might be a promising therapeutic for patients with cholesterol pneumonia.     

Interventional re‐opening of a PDA for reverse potts shunt circulation after ADO I implantation in a child

We report interventional re‐opening of a PDA for reverse Potts shunt circulation 12 months after closure in a 3.8 year old child, suffering from right ventricular (RV) failure due to suprasystemic pulmonary artery hypertension (PAH) after ADO I implantation. After ex vivo simulation, perforation through the mesh of the ADO I with the use of transseptal needle, wire looping (AO‐PA), balloon dilatation, stent implantation (Palmaz 6 mm) and post dilatation, a reverse Pott‐shunt circulation was established. A follow‐up period of 11 months was achieved with preserved RV function and reverse Pott shunt circulation maintained a post ductal saturation of 94–88%. © 2016 Wiley Periodicals, Inc.     

Simultaneous stenting of tightly stenosed patent ductus arteriosus and pulmonary artery bifurcation using two stents (Y stenting): An innovative technique

Stenting of patent ductus arteriosus (PDA) is a palliative technique that is evolving as an alternative to shunt surgery. Patients with duct‐dependant pulmonary circulation and branch pulmonary artery stenosis are often palliated by shunt surgery with repair of branch pulmonary arteries under cardiopulmonary bypass. We present here an 8‐month‐old male child with duct‐dependant pulmonary circulation with bifurcation stenosis who was palliated successfully by transcatheter means. He had stenosed PDA with tight pulmonary artery bifurcation stenosis and underwent successful “Y” stenting of PDA with simultaneous deployment of two stents. He successfully underwent bidirectional Glenn surgery 8 months after the procedure. Simultaneous stenting of bifurcation stenosis of branch pulmonary arteries with two stents has not been described in the literature. © 2014 Wiley Periodicals, Inc.     

Patent ductus arteriosus balloon sizing: A new technique to evaluate the size in complex cases

Patent ductus arteriosus (PDA) transcatheter closure is a widespread procedure. However in some cases PDA measurements may be unclear and choice of the proper device could be quite difficult. This may happen in large PDA and in particular in adults. We have developed a new technique using an ASD sizing balloon to measure the PDA in order to better understand PDA anatomy and size. The first step is to create an artero‐venous circuit across the PDA. A 24 or a 34 mm Amplatzer balloon sizing for ASD closure is placed over the wire from the venous access in the descending aorta. Then, the balloon is inflated and gently pulled back across the PDA toward the pulmonary artery. The frame where the balloon is exactly across the PDA is chosen and measurements performed. In conclusion, a new method for PDA measurement in large PDA is reported. The procedure is safe and reliable. © 2015 Wiley Periodicals, Inc.     

Effect of patent ductus arteriosus on pulmonary vascular disease

The hemodynamic effects of a patent ductus arteriosus (PDA) are well known including systemic hypoperfusion and volume overload on the left ventricle. This article aims to provide a review of the long‐standing effect of a hemodynamically significant PDA on the pulmonary vasculature and the role of cardiac catheterization in preterm infants with a PDA and pulmonary hypertension.     

Aortopulmonary Window in Adults: Diagnosis and Treatment of Late‐presenting Patients

Objectives. Aortopulmonary window is an uncommon condition, particularly so in adulthood because it is usually fatal in infancy or childhood if untreated. Very few cases of those who have survived to adulthood and been operated on successfully have been described. Our study aimed to provide clinical, investigative, surgical, and outcome details of such patients. Design and Setting. Retrospective study of consecutive adult patients with aortopulmonary window treated at a tertiary charitable cardiovascular institute in South India between 1996 and 2006. Results. Six adult patients successfully underwent aortopulmonary window closure. Five of the six patients had been correctly diagnosed on echocardiography, while one was only diagnosed after cardiac catheterization for unexplained pulmonary arterial hypertension. Four of the patients had large defects with severe pulmonary arterial hypertension, with pulmonary vascular resistance index (PVRI) ranging from 5.2 to 15.9 at baseline. All showed significant reversibility with oxygen administration, with PVRI on oxygen falling to between 0.6 and 2.2. These patients successfully underwent cardiopulmonary bypass. The other two patients with small lesions underwent ligation off‐pump. There was no early or late mortality among these patients. All were in New York Heart Association class I, on follow‐up ranging from 3 months to 8 years. Conclusions. Aortopulmonary window may rarely present in adulthood. The diagnosis can usually be made by careful echocardiography alone. Even in the presence of severe pulmonary arterial hypertension, if a significant reversibility in pulmonary vascular resistance can be demonstrated with oxygen, the condition can be successfully corrected with good long‐term outcomes.     

Rosiglitazone attenuates hypoxia‐induced pulmonary arterial hypertension in rats

Background and objective: Expression of peroxisome proliferator‐activated receptor gamma (PPARγ) is decreased in the lungs of patients with pulmonary hypertension, and PPARγ ligands have been associated with the release of vasoactive substances from vascular endothelial cells and prevention of vascular remodelling. We hypothesized that PPARγ may play a critical role in the development of pulmonary hypertension induced by chronic hypoxia. Methods: Male adult Sprague‐Dawley rats were exposed to normoxia, normoxia and rosiglitazone (8 mg/kg orally, 5 days/week), hypoxia (12% inspired O₂ fraction), or hypoxia and rosiglitazone for 4 weeks. On the last day of the fourth week, pulmonary arterial pressure was measured and morphological changes in pulmonary vessels were assessed. The expression of PPARγ, endothelin (ET)‐1 and vascular endothelial growth factor (VEGF) was also analysed. Results: Rosiglitazone inhibited the development of pulmonary hypertension, and pulmonary vascular remodelling induced by chronic hypoxia. PPARγ expression was decreased and expression of ET‐1 and VEGF was increased in lung tissues of the hypoxia group. Rosiglitazone treatment prevented the hypoxia‐induced reduction in PPARγ expression, and restored ET‐1 and VEGF expression almost to the levels of the normoxia group. Conclusions: Rosiglitazone inhibited the development of pulmonary hypertension induced by chronic hypoxia, perhaps by reversing the changes in PPARγ, ET‐1 and VEGF expression induced by hypoxia. These findings indicate that rosiglitazone may be beneficial in the treatment of chronic hypoxic pulmonary hypertension.     

A novel therapeutic approach in pulmonary arterial hypertension as a complication of adult‐onset Still's disease: targeting IL‐6

Adult‐onset Still's Disease (AOSD), often though as the adult variant of systemic juvenile idiopathic arthritis (JIA), has an incidence of 1–3 cases per 1 million. Cardinal manifestations include fever, arthritis, skin rash, sore throat, hepatosplenomegaly and lymphadenopathy. Prolongation in diagnosing this disease results from its similarity to infectious, malignant and rheumatic diseases and lack of biomarkers. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only six cases reported in literature to date. Here we present a patient with AOSD who has developed pulmonary hypertension as a complication. We report a case of AOSD complicated by PAH treated successfully with tocilizumab, a humanized monoclonal antibody to human interleukin (IL)‐6 receptor. A Pubmed and Medline search for evidence of pulmonary hypertension in AOSD and use of IL‐6 inhibition in management was performed. Data for this study was collected from the patient's chart records. No infectious or neoplastic cause of her disease was identified and after extensive diagnostic workup, the patient was diagnosed with AOSD fulfilling Yamaguchi criteria. After initiation of IL‐6 therapy the patient was followed over time to monitor the hemodynamic changes in pulmonary vasculature. Following treatment with Tocilizumab, the patient showed dramatic improvement in her clinical symptoms and remains in remission, through combination of tocilizumab (8 mg/kg), methotrexate and prednisone. Improvement of systemic symptoms, right heart catheterization (RHC) findings and the VECTRA‐DA score served as a measure of treatment response. Tocilizumab has been effective in demonstrating marked improvement in both the clinical and laboratory parameters. Tocilizumab is an effective novel treatment for AOSD with PAH. This is the first documented report of successful use of tocilizumab in AOSD patients presenting with PAH. Prospective comparative studies could help validate its efficacy and safety.     

Patients with Pulmonary Hypertension Related to Congenital Systemic‐to‐Pulmonary Shunts are Characterized by Inflammation Involving Endothelial Cell Activation and Platelet‐mediated Inflammation

Objective. We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts and the change in these markers during treatment with bosentan. Background. Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts is largely unknown. Patients and Methods. Plasma levels of several inflammatory mediators were determined by enzyme immunoassays in 14 children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts before and after 12 months treatment with bosentan, and compared with levels in 54 healthy controls. Results. The patients were characterized by increased plasma levels of von Willebrand factor (～2.5‐fold), C‐reactive protein (～3.5‐fold), and soluble CD40 ligand (～2.5‐fold) as compared with controls, representing markers of endothelial cell activation, systemic inflammation, and platelet‐mediated inflammation, respectively. Patients also had significantly elevated plasma levels of osteoprotegerin (～1.6‐fold). Within the study group, N‐terminal pro‐brain natriuretic peptide levels correlated significantly with the concentrations of C‐reactive protein (r= 0.61, P < .027) and von Willebrand factor (r= 0.74, P= .004). Except for a decline in monocyte chemoattractant protein‐1 and receptor activator of nuclear factor‐κB ligand, bosentan therapy did not attenuate the systemic inflammation. Conclusion. Children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts are characterized by enhanced systemic inflammation involving increased endothelial cell activation and platelet‐mediated inflammation. These inflammatory responses seem essentially to be unmodified by bosentan, potentially representing new targets for therapy in this disorder.