Anatol J Cardiol 2016; 16: 63-7 Case Reports
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Recommendations regarding specific anticoagulation therapy after device implantation remain controversial (9). It is accepted that 6 months of aspirin alone is usually effective in preventing early thrombus formation on the device. However, there was one patient with nonendothelialization of the left atrial disk 32 months after ASO device placement (10). Heparin, at a dose of 100 U/kg during implantation, was given to the patient, and aspirin alone was used to prevent thrombosis for 6 months since our patient had no history of pre-thrombotic event. Also, there was no coagulation dis-orders detected in our patient. Nevertheless, huge thrombus was detected at the central part of the left side of the well endothelial-ized Amplatzer device after 1 year of implantation. Therefore, ad-ditional long-term follow-up studies are needed to reevaluate the duration or type of anticoagulation in children with closure devices.
Although TEE was shown to be more sensitive than TTE in detecting thrombus formation in adults, follow-up by TTE as an imaging perspective might be sufficient for younger children. We also preferred to follow-up all children with closure devices by TTE because of good echocardiographic windows. For this rea-son, it is not clear whether the thrombus was present at the early period after device implantation with TEE imaging. Therefore, it was emphasized that more studies are required to determine the choice of imaging method after device implantation in children with ASDs even with a good quality of transthoracic imaging.
Conclusion
To the best of our knowledge, this is the first reported case of a child with late huge thrombus on an Amplatzer device without any known risk factor. Additional longer follow-up studies are warranted in children to determine the duration and the type of antiplatelet therapy and the preference of imaging technique af-ter device implantation.
References
1. Cooke J, Gelman J, Menahem S, Harper RW. Thrombus on an ASD clo-sure device: a call for caution. Heart, Lung Circ 2000; 9: 30-1. [CrossRef]
2. Krumsdorf U, Ostermayer S, Billinger K, Trepels T, Zadan E, Horvath K, et al. Incidence and clinical course of thrombus formation on atri-al septatri-al defect and patent foramen ovatri-ale closure devices in 1000 consecutive patients. J Am Coll Cardiol 2004; 43: 302-9. [CrossRef]
3. Bonou M, Lampropoulos KM, Barbetseas J. Thrombus formation 10 years after placement of an atrial septal secundum defect closure device. Eur Heart J 2012; 33: 704. [CrossRef]
4. Majunke N, Bialkowski J, Wilson N, Szkutnik M, Kusa J, Baranows-ki A, et al. Closure of atrial septal defect with the Amplatzer septal occluder in adults. Am J Cardiol 2009; 103: 550-4. [CrossRef]
5. Cetta F, Arruda MJ, Graham LC. Large left atrial thrombus formation despite warfarin therapy after device closure of a patent foramen ovale. Cathet Cardiovasc Intervent 2003; 59: 396–8. [CrossRef]
6. Snijder RJ, Suttorp MJ, Berg JM, Post MC. Percutaneous closure of secundum type atrial septal defects: More than 5-year follow-up. World J Cardiol 2015; 7: 150-6. [CrossRef]
7. Sherman JM, Hagler DJ, Cetta F. Thrombosis after septal closure device placement: a review of the current literature. Catheter
Car-diovasc Interv 2004; 63: 486-9. [CrossRef]
8. Olabiyi OO, Morales DL, Franklin WJ. De novo thrombus on an atrial septal defect device 3 years after its implantation. Pediatr Cardiol 2013; 34: 1269-71. [CrossRef]
9. Khairy P, O’Donnell CP, Landzberg MJ. Transcatheter clsoure versus medical therapy of patent foramen ovale and presumed paradoxi-cal thromboemboli. Ann Intern Med 2003; 139: 753–60. [CrossRef]
10. Chessa M, Butera G, Frigiola A, Carminati M. Endothelialization of ASD devices for transcatheter closure: possibility or reality? Int J Cardiol 2004; 97: 563-4. [CrossRef]
Video 1. Transthoracic echocardiography of the patient with huge thrombus on the left atrial disk of the device (paraster-nal long-axis view).
Address for Correspondence: Dr. Fahrettin Uysal
Uludağ Üniversitesi Tıp Fakültesi, Pediyatrik Kardiyoloji Bölümü, Görükle Kampüsü, 16059 Nilüfer, Bursa-Türkiye
Phone: +90 224 295 04 49 Mobile: +90 505 476 95 86 Fax: +90 224 442 81 43 E-mail: fahrettin_uysal@mynet.com Accepted Date: 27.10.2015
©Copyright 2016 by Turkish Society of Cardiology - Available online at www.anatoljcardiol.com
DOI:10.14744/AnatolJCardiol.2015.6538
Introduction
Pulmonary hypertension (PH) is frequent among patients with β-thalassemia intermedia (TI) and β-thalassemia major (TM) (1). Almost 60% of all TI patients develop PH (2). However, no ran-domized controlled trials have evaluated this condition-specific treatment options. Recent guidelines for the treatment of PH of-fer no specific recommendations for these patients; moreover, the classification of chronic hemolytic anemia was changed from group I PH to group V PH, in which group pulmonary ar-terial hypertension (PAH)-specific therapy is not recommended (3). We report three patients with β-TI who developed severe PH and were successfully treated with PAH-specific therapies.
Case Reports
Case 1A 39-year-old man with β-TI was admitted with new-onset dyspnea and fatigue. On further examination, systolic pulmonary arterial pressure (sPAP) was measured as 115 mm Hg on
trans-Treatment of pulmonary hypertension
in three patients with β-thalassemia
intermedia using pulmonary arterial
hypertension-specific medications
Demet Menekşe Gerede, Aynur Acıbuca, Tamer Sayın, Çetin Erol Department of Cardiology, Faculty of Medicine, Ankara University; Ankara-Turkey
Case Reports
thoracic echocardiography (TTE). The right heart catheterization (RHC) was performed to confirm the diagnosis of PH. It showed sPAP, pulmonary artery diastolic pressure (dPAP), and mean pul-monary artery pressure (sPAP) of 70, 38, and 49 mm Hg, respec-tively. The pulmonary capillary wedge pressure (PCWP) was 11 mm Hg. Treatment with bosentan (62.5 mg bid) and sildenafil (25 mg tid) was initiated. He has been followed-up in our outpatient clinic during the past 2 years and his sPAP decreased to 45 mm Hg after the first year of therapy.
Case 2
A 52-year-old man with β-TI and PH was admitted because of progressive dyspnea under sildenafil. His sPAP was measured as 80 mm Hg on TTE. The RHC showed sPAP, dPAP, and sPAP of 79, 29, and 45 mm Hg, respectively. The PCWP was 13 mm Hg. Inhaled iloprost (20 mcg q4h) was added, and the dosage of sildenafil was increased (25 mg qid). Two years after discharge, he was drecom-pensated, and a control TTE showed a sPAP of 95 mm Hg. The dos-age of iloprost was increased to 20 mcg q3h, while the dosdos-age of sildenafil was raised to 80 mg tid. After 1 year of this therapy, PH (sPAP: 80 mm Hg) and functional limitations were persistent. There-fore, bosentan (125 mg bid) was initiated, and sPAP decreased to 65 mm Hg; after 3 months, symptomatic relief was observed.
Case 3
A 51-year-old woman with β-TI presented with dyspnea and palpitations. During the further investigation, TTE revealed PH (sPAP: 80 mm Hg). After excluding other possible etiologies, the patient was assumed to have thalassemia-related PH. Because of her poor health condition and thrombocytopenia, she was un-able to undergo RHC. After 3 months of therapy with sildenafil (20 mg tid) and an iloprost inhaler (20 mcg q3h), her symptoms and 6-min walk distance (6MWD) improved. Also, the sPAP de-creased to 60 mm Hg after 1 year of dual PAH-specific therapy. After 5 years of this treatment, which provided a favorable func-tional capacity for her, she died following pneumonia.
Discussion
The pathophysiology of PH in thalassemia is multifactorial, and treatment may be based upon these factors. Chronic hemolysis causes PH by inducing nitric oxide (NO) and arginine deficiency (4). Low NO bioavailability causes endothelial dysfunction, which leads to inflammation, vasoconstriction, and hypercoagulopathy (5). Published data indicate that transfusion therapy decreases chronic hemolysis and also chronic tissue hypoxia, which is a risk factor for PH (6). Unlike patients with TM, those with TI do not require regular transfusions. Our three patients only received transfusions intermittently as needed. Therefore, the protective effect of regular transfusion was not probable.
Case reports have shown that PAH-specific drugs might be useful in patients with thalassemia. A 12-week prospective study of sildenafil that enrolled 10 patients with β-thalassemia
and PH revealed an improvement in New York Heart Associa-tion (NYHA) class and a 13% reducAssocia-tion in tricuspid regurgitaAssocia-tion velocity (7). A case report described an improvement in pulmo-nary hemodynamics after 1 year of bosentan therapy in a patient with TI-related PH (8). Tam et al. (9) showed that intravenous epoprostenol improved symptoms and reduced mPAP in a TM patient with PH. In addition, an increase in 6MWD, a decrease in PAP, and an improvement in the NYHA class and function of right ventricle were shown following the continuous infusion of epoprostenol in a patient of β-TI and PH (10).
We recorded symptomatic relief, a decrease in sPAP, and an increase in 6MWD in all three patients with a combination of PAH-specific medications. However, these improvements cannot be at-tributed to a single agent due to the concomitant use of these drugs.
Conclusion
This case reports support the use of PAH-specific medica-tions in this group. To the best of our knowledge, this is the first case report to show a possibrle benefit of treatment with inhaled prostaglandin. However, further studies will be needed to deter-mine the best treatment for PH in patients with β-thalassemia.
References
1. Aessopos A, Stamatelos G, Skoumas V, Vassilopoulos G, Mantzourani M, Loukopoulos D. Pulmonary hypertension and right heart failure in pa-tients with beta-thalassemia intermedia. Chest 1995; 107: 50-3. [CrossRef]
2. Chueamuangphan N, Patumanond J, Wongtheptien W, Nawara-wong W, Sukonthasarn A, Chuncharunee S, et al. Benefits of chron-ic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension. Int J Gen Med 2014; 7: 411-6. [CrossRef]
3. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Gho-frani A, et al. Updated clinical classification of pulmonary hyper-tension. J Am Coll Cardiol 2013; 62: D34-41. [CrossRef]
4. Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med 2004; 350: 857-9. [CrossRef]
5. Gladwin MT, Kato GJ. Cardiopulmonary complications of sickle cell disease: role of nitric oxide and hemolytic anemia. Hematology Am Soc Hematol Educ Program 2005; 51-7. [CrossRef]
6. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 2001; 97: 3411-6. [CrossRef]
7. Morris CR, Kim HY, Wood J, Porter JB, Klings ES, Trachtenberg FL, et al. Sildenafil therapy in thalassemia patients with Doppler-defined risk of pulmonary hypertension. Haematologica 2013; 98: 1359-67. [CrossRef]
8. Anthi A, Tsangaris I, Hamodraka ES, Lekakis J, Armaganidis A, Or-fanos SE. Treatment with bosentan in a patient with thalassemia intermedia and pulmonary arterial hypertension. Blood 2012; 120: 1531-2. [CrossRef]
9. Tam DH, Farber HW. Pulmonary hypertension and beta-thalas-semia major: report of a case, its treatment, and a review of the literature. Am J Hematol 2006; 81: 443-7. [CrossRef]
10. Ussavarungsi K, Burger CD. Pulmonary arterial hypertension in a patient with β-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker thera-py: review of literature. Pulm Circ 2014; 4: 520-6. [CrossRef]
Anatol J Cardiol 2016; 16: 63-7 Case Reports
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Address for Correspondence: Dr. Demet Menekşe Gerede Ankara Üniversitesi Tıp Fakültesi, Cebeci Kalp Merkezi, 06590 Cebeci; Ankara-Türkiye
Phone: +90 312 595 62 86 Mobile: +90 532 599 10 91 Fax: +90 312 363 22 89 E-mail: drmeneksegerede@yahoo.com Accepted Date: 20.08.2015
©Copyright 2016 by Turkish Society of Cardiology - Available online at www.anatoljcardiol.com
DOI:10.14744/AnatolJCardiol.2015.6624
Introduction
Pneumopericardium is defined as the accumulation of air in the pericardial cavity (1). Many cases have since been reported, mostly due to blunt or penetrating chest injuries in adults. Spon-taneous pneumopericardium is seen very rarely (2). The mortality rate for patients with pneumopericardium who present with symp-toms of tamponade is 50% (3). We present the case of a 27-year-old 32-week-pregnant woman who died due to pneumopericardium.
Case Report
A 27-year-old 32-week-pregnant woman was admitted to the emergency department with a 3-day history of shortness of breath and chest pain. She did not define any history of trauma or surgery. On physical examination, body temperature was 36.6°C. Heart rate, blood pressure, and respiratory rate were 125 beat/min, 90/55 mm Hg, and 33/min, respectively. Tachycardia and decreased heart sounds were detected on cardiovascular examination; at the same time, crackle and splashing mill-wheel sounds were also ob-served. Bilaterally decreased breath sounds and subcrepitant rale were prominent in respiratory system. Electrocardiography (ECG) revealed sinus tachycardia. White blood cell count and C-reactive protein were 22000 mm3 and 7.22 mg/dL, respectively. Other labo-ratory parameters were within normal limits. In the examination by the obstetrician, intrauterine death was determined. A radiolucent area surrounding the heart was observed on chest X-ray (halo sign) (Fig. 1). Transthoracic echocardiography in the emergency department revealed air bubbles in the pericardial cavity (swirling air sign) together with the systolic echo signal loss. There was no signal loss in the epicardium and chest layers (Video 1). Thoracic computed tomography showed pneumopericardium and
dial effusion (Fig. 2). In the fluoroscopic imaging-guided pericar-diocentesis that was performed under emergency conditions due to deep hypotension, 300-mL foamy fluid with air was removed. Fluid was exudate on laboratory examination. There was no bac-terial growth on culture. Adenosine deaminase was negative, and cytological investigation of the fluid was benign. Secondary to continuing respiratory distress, the patient was intubated. Positive inotropic therapy was started. No improvement was detected in hypotension, and cardiopulmonary arrest occurred. Cardiopulmo-nary resuscitation failed, and the patient died.
Spontaneous pneumopericardium in
a pregnant woman
Adil Bayramoğlu, Zeki Yüksel Günaydın, Osman Bektaş, Ahmet Karagöz1, Yılmaz Ömürotlu2
Department of Cardiology, Faculty of Medicine, Ordu University; Ordu-Turkey
1Department of Cardiology, Faculty of Medicine, Giresun University; Giresun-Turkey
2Clinic of Cardiology, Kars State Hospital; Kars-Turkey
Figure 1. A radiolucent band of air completely surrounding the heart (halo sign)
