• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.216-220
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• 2005

Pulmonary arteriovenous fistulas(PAVFs) is a rare disorder that occurs in two to three children per 100,000 population. It is presented as absence of intervening capillary beds between the pulmonary artery and vein with resultant persistent right to left shunt. Other causes include trauma, liver cirrhosis, malignancy and schistosomiasis. It is mostly asymptomatic, but it may present with respiratory difficulty, cyanosis, clubbed fingers induced by right to left shunt or hemoptysis, polycythemia and epistaxis. Major complications, such as brain abscess, brain embolism, paradoxical embolism and subacute infective endocarditis can be devastating, so therapeutic intervention is recommended in all patients. However, removal of low-resistance fistulas can aggrevate pulmonary hypertension, so detection of increased pulmonary pressure is important. We report two patients : One a 42 year-old male with PAVFs treated with coil embolization, and a 42 year-old female who was treated with anticoagulants due to pulmonary hypertension.

• Journal of the Korean Society of Radiology
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• v.84 no.6
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• pp.1378-1383
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• 2023

Pulmonary artery sarcomas are rare, high-grade malignancies, primarily affecting the proximal elastic pulmonary artery and usually manifesting as tumoral impaction on imaging. Due to similar clinical and imaging findings, pulmonary artery sarcomas are frequently misdiagnosed as pulmonary thromboembolism or, occasionally, as vasculitis. Herein, we reported a case of pulmonary artery intimal sarcoma initially misdiagnosed as pulmonary thromboembolism and vasculitis due to its relatively atypical location and morphology, along with a literature review.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.199-207
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• 2002

Purpose : Since the successful application of total atrio-pulmonary connection(TAPC) to patients with various types of physiologic single ventricles in 1971, post-operative survival rates have reached more than 90%. However some patients have been shown to present with late complications such as right atrial thrombosis, atrial fibrillation and protein losing enteropathy eventually leading to re-operation to control the long-term complications. The aim of this study is to review the results of total cavo-pulmonary connection(TCPC) in cases with late complications after TAPC. Methods : Between Jan. 1995 and Dec. 2000, 6 patients(5 males and 1 female) underwent cardiac catheterization $11{\pm}3$ months after conversion of previous TAPC to TCPC. We compared the hemodynamic and morphologic parameters before and after TCPC and also assessed the clinical outcomes. The indications for TAPC were tricuspid atresia in 4 cases and complex double-outlet right ventricle with single ventricle physiology in 2 cases. Results : There was no peri-operative mortality and all patients were clinically and hemodynamically improved at a mean follow-up of 11 months(range : 4 to 13). However, protein losing enteropathy recurred in 2 patients; this was were successfully treated with subcutaneous administration of heparin. Right atrial pressure before TCPC was $18.0{\pm}3.6mmHg$, but baffle pressure, corresponding to right atrial pressure decreased to $14.8{\pm}3.6mmHg$ after TCPC. The size of the pulmonary arteries did not regress after TCPC. Conclusion : The conversion of TAPC to TCPC improves clinical and hemodynamic status by decreasing the right atrial pressure and by providing a laminar cavo-pulmonary flow which enhances the effective pulmonary circulation in the so-called Fontan circulation.

• Journal of Chest Surgery
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• v.29 no.6
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• pp.639-645
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• 1996

A 14-year-old male patient with previous surgical repair of tetralogy of Fallot was admitted with hemodynamically significant ventricular tachycardia (VT). On preoperative electrophysiologic study (EPS), the morphology of documented VT was RBBB of vertical axis with 320 msec cycle length. The endocardial mapping during VT delineated the origin of VT at right ventricular outflow tract (RVOT), where the patch was attached. The clinical VT had a clockwise reentry circuit around the patch with the earliest activation at the same site seen during the preoperative EPS. The previously placed right ventricular outflow patch and fibrous tissue were removed. During a postoperative EPS, it was no longer possible to induce the VT. Ventricular tachycardia following repair of tetralogy of Fallot seen in this patient was caused by a macro-reentry around the right ventricular outflow patch. We were able to ablate the VT with the aid of a detailed mapping of its epicardial activation sequence.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1306-1315
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• 1996

This study reviewed the changes of hemodynamlcs and centrAl pulmonary artery dimension in 54 patients who underwent bidirectional cavopulmonary shunt(BCPS) between February 1992 and December 1995 at Seoul National University Childrell's Hospital. Ag and body weight of patients averaged 36.8 $\pm$ 37.7 months and 8.0$\pm$3.0 kg, respectively Eightynine percent of patients had more than 2 violations of the risk factors for Fontan operation, resulting overall hospital mortality of 16.6%(9154). Serial hemodynamic and anglographic examinations before and mean 16.3 $\pm$ 14.3 months iirter BCPS were compared. The arterial oxygen saturation improved from a preoperative value of 71 9: 10.1 % to 79.H $\pm$ 8. 5% (n:4), p<0.05). The values of arterial oxygen saturation were lower as the age of the patients with BCPS in place was older(n=22, R'=0.341, p=0.004). A mean pulmonary artery pressure and pulmonary vascular resistance reduced from 31 $\pm$17 to 1).5$\pm$3.SmmHg(n=22, p<0.05) and from ).2$\pm$2.1 to 2.3$\pm$2.7 unit (n=7. p>0.05), respectively. Follow-up study showed a significant Increase of absolute values of ipsilateral pulmoanry artery (n: 14, p<0.05), but no change of contralateral pulmonary artreries (n: 14. p=not significant(HSI). However, there w re significant decreases in diameters of both ipsilateral and contralateral pulmonary arteries standardized by patients' body surface areas(16.8% decrease, n: 14, p< 0. 05 for ipsilatreal, 25.1%, n=14, p<0.05 for contralateral). Pulmonary artery indices for cross sectional areas of both pulmonary arterises decreased 9.3 $\pm$ 13.8% with showing a trend of more decrease as the follow-up duration was longer, We conclude that the bidirectional cavopulmonary shunt provide an excellent.

• Journal of Chest Surgery
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• v.37 no.7
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• pp.597-600
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• 2004

We report a rare case of pulmonary artery sarcoma mimicking pulmonary artery thromboembolism in a 57-year-old man who suffered with 2-month dyspnea and exacerbated for 1 week. He was transferred from private clinic and he was diagnosed as acute pulmonary artery thromboembolism on the basis of chest CT. Chest CT, pulmonary artery angiogram, and perfusion scan were examined. We performed surgical excision with aid of CPB. The final pathologic report was that the mass was a pulmonary artery sarcoma. We experienced one case of pulmonary artery sarcoma and reported it with reference.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.552-555
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• 2001

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.430-434
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• 2008

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.350-355
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• 2002

Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 2010

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.