Long-term follow-up and outcomes of discrete subaortic stenosis resection in children.

BACKGROUND: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. MATERIALS AND METHODS: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. RESULTS: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25-13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1-13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67-16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1-9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. CONCLUSIONS: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Unusual survival time of primary cardiac sarcoma of the right ventricle.

Primary cardiac sarcomas are rare tumors with unfavorable prognosis. We report a 69-year-old male with a right ventricular mass diagnosed as primary malignant cardiac sarcoma with unexpected long survival of 16.5 months.

The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy.

OBJECTIVES: We sought to evaluate the impact of interruption of anomalous systemic arterial supply (ASAS) on clinical course and management outcome of scimitar syndrome (SS) presenting during infancy. BACKGROUND: No systematic study has been reported so far on this subject, although there are conflicting sporadic reports indicating variable effect. METHODS: Out of 23 children diagnosed to have SS during the past 25 years, 16 symptomatic infants had ASAS. After interrupting ASAS (coil embolization -14, surgical ligation -2), they were prospectively followed up to define their clinical course and management outcome. RESULTS: All 16 infants had sizable ASAS and 9 had variable scimitar vein (SV) stenosis. Fifteen (94%) had pulmonary hypertension and significant left to right shunt. Post intervention, there was variable reduction of shunt in 14 and pulmonary artery pressure in 15 cases. All showed varying clinical improvement. One died of septicemia shortly afterwards. Definitive surgery was deferred for optimal results in seven children for a mean period of 8 months (range 1 month to 3 years). Remaining eight children did not require definitive surgery. One among them had closure of stenosed partial SV by Amplatzer duct occluder ("physiologic correction"). Two children on short term and six children on long term follow-up (mean 5.2 years; range 3.3-10.3 years) are doing well. CONCLUSION: Interruption of ASAS helps to avoid or defer definitive surgery for SS during infancy. Therefore, we recommend coil embolization of ASAS as initial palliation, and long term surveillance to assess need for further intervention.

Transcatheter closure of large pulmonary arteriovenous fistula including pulmonary artery to left atrial fistula with Amplatzer septal occluder.

OBJECTIVES: We sought to evaluate the safety and efficacy of Amplatzer septal occluder (ASO) to close very large pulmonary arteriovenous fistula (PAVF). BACKGROUND: Transcatheter coil embolization, the standard treatment for PAVF, has potential complications particularly in patients with very large fistulas. Several recently available devices have been tried effectively, however, they too have limitations. METHODS: During 2004, three patients (aged 17-56 years), diagnosed with large PAVF including one with pulmonary artery (PA) to left atrial (LA) fistula, had interventional closure prospectively using ASO. Following hemodynamic evaluation and angiographic localization of PAVF, the feeding artery (FA) was selectively cannulated with Amplatzer delivery sheath. ASO, with the right atrial (RA) disc diameter equal to or up to 4 mm larger than the maximum diameter of FA, was delivered through it in such a way that the left atrial disc assumed an oblong shape and the right atrial disc retained its designed flat configuration for better anchorage and thrombogenicity. RESULTS: All patients had very large PAVF fed by a single FA (size 12-24 mm), which was closed completely, without complications, using ASO (size 7-16 mm; RA disc diameter 15-26 mm). Their arterial saturation rose from mean 72.3% to 97.3%. Follow up (1.5-3 years) showed disappearance of the radiological shadows and stable arterial saturations (mean 97.7%). CONCLUSIONS: We conclude that, using the new criteria for device size selection and modified technique of implantation, very large PAVFs including PA to LA fistula can be closed safely, effectively and nonsurgically with ASO.

Pulmonary arteriovenous malformation presenting with severe hypoxemia.

OBJECTIVE: To report a case of right pulmonary arteriovenous malformation (PAVM) affecting the right upper lobe, following the incidence of empyema thoracis in the contralateral lung. CLINICAL PRESENTATION AND INTERVENTION: A 19-year-old, previously healthy male presented with acute respiratory distress, left pleuritic chest pain, fever and hypoxemia. Clinical findings, laboratory and radiological examinations including pulmonary angiogram were consistent with the diagnosis of left pneumonia complicated with parapneumonic pleural effusion and right upper lobe PAVM. The patient was intubated and ventilated because of persistent hypoxemia. He was successfully treated by percutaneous transcatheter embolization. CONCLUSION: This case shows that percutaneous transcatheter embolization is a safe and effective first option for the treatment of PAVM.

Tuberculous pericardial effusion: features, tamponade, and computed tomography.

The clinical features with particular reference to tamponade and mediastinal adenopathy were studied in tuberculous pericardial effusion. Tamponade is a frequent complication and the recognition of tuberculous etiology can be difficult. Involvement of the pericardium is mostly from mediastinal lymph nodes that have not been studied. This was a prospective cohort study. All patients had large effusions, and underwent pericardiocentesis and chest computed tomography. Patients with tuberculosis had specific therapy. Others with viral/idiopathic effusion served as controls for the computed tomography studies. There were 26 patients with tuberculosis: 18 had tamponade on echocardiography. All had symptoms. Fever (n = 23) and dyspnea (n = 20) were the most frequent presenting symptoms. Pericardial rub was heard in 14, and 3 had enlarged cervical or axillary nodes. Pulmonary tuberculosis was present in 6. Tuberculin skin test measured 17 +/- 3.3 mm. The biopsy specimen showed a granuloma in 22 of 24. All 26 had mediastinal lymph nodes > 10 mm with a mean size of 19.5 +/- 8.6 mm that disappeared (81%) or regressed (19%) on treatment (p < 0.001). Aortopulmonary nodes were most frequently enlarged (65.4%) and hilar the least. Three required pericardiectomy. At follow-up all were doing well. None with viral/idiopathic effusion had lymph node enlargement. Fever, dyspnea, and tamponade were frequent with tuberculosis. The prognosis was good with specific therapy. Mediastinal nodes were enlarged in all and only with tuberculosis and not with viral/idiopathic effusion. Nodes disappeared or regressed with treatment. In the appropriate clinical context, mediastinal lymph node enlargement on chest computed tomography along with a strongly positive skin test results could help in the diagnosis of a tuberculous etiology of pericardial effusion.

Percutaneous multiple-site parietal pleural biopsy: description and evaluation of a new and safe technique.

STUDY OBJECTIVES: (1) To describe a new percutaneous pleural biopsy technique to obtain multiple-site parietal pleural biopsy specimens in patients with pleural effusion (PE), and (2) to evaluate its effectiveness and safety compared to current techniques. DESIGN: Prospective interventional study. SETTING: University teaching hospital. PATIENTS: Consecutively referred for evaluation of exudative PE. INTERVENTION: With the patient in a semirecumbent position, a 9F sheath was inserted by the Seldinger technique into the pleural cavity on the midaxillary line under local anesthesia and fluoroscopic guidance. An 8F bioptome was introduced through it, and biopsy specimens were taken from several sites on the costal and diaphragmatic pleura. After biopsy, PE was completely evacuated, and the sheath was removed. RESULTS: During the 2-year pilot study, we procured, on average, 14 adequate pleural specimens from each of the 28 patients (age range, 15 to 81 years) on the first attempt. Histopathologic examination revealed tuberculous pleuritis (18 patients), metastatic adenocarcinoma (1 patient), and nonspecific pleuritis (9 patients). Postprocedure, 25 patients had rapid symptomatic improvement without recurrence of PE. No major complications occurred during or after the procedure (mean follow-up period, 2 years). CONCLUSIONS: Our new biopsy procedure can be performed easily, safely, and with increased diagnostic sensitivity and patient comfort. Unlike other biopsy techniques, it provides adequate multiple-site pleural biopsy specimens, in all cases, on the first attempt without any morbidity and mortality. It has a therapeutic potential to provide rapid symptomatic relief and treatment by pleurodesis. We recommend this procedure for patients whose conditions remain undiagnosed after undergoing needle biopsy or for those who cannot tolerate it, before considering more aggressive diagnostic interventions. This is the best alternative when thoracoscopy or thoracotomy are not available or when patients are at high risk for complications from them.

Transcatheter coil closure of pulmonary arteriovenous malformations in children.

Pulmonary arteriovenous fistula (PAVF) is a rare vascular malformation of the lung that may lead to cyanosis, epistaxis, hemoptysis, and neurological deficits or cerebral abscess. The purpose of this study is to assess the effectiveness of percutaneous transcatheter embolization of PAVF in pediatric patients. Transcatheter embolization of PAVF using spring coils was performed in three patients (two males and one female) who presented between 1989 and 1999. The age at presentation ranged from 8 months to 3 years (mean 19.6 months). All patients had cyanosis and clubbing. Neurological, dermatological, or other cardiac manifestations were absent. The arterial oxygen saturations at presentation ranged from 60 to 72% (mean 64%). During eight procedures, multiple coils (total of 41 coils, average 14 coils per patient) were delivered to occlude the fistulas successfully. There was complete occlusion of the fistulas in all patients after the multiple interventions. The aortic saturations increased from a mean of 66% to a mean of 95%. Chest radiographs demonstrated dramatic regression of the pulmonary shadows in all three patients. There were no complications encountered during the procedures or during follow-up. Transcatheter coil embolization of PAVF is a safe and effective method of treatment in the pediatric age group. Patients may require multiple procedures to completely occlude the fistulas. Long-term follow-up is essential to ensure absence of recurrence due to recanalization.

Tuberculous pericardial effusion--mediastinal lymph glands: the cause and clue to the etiology.

BACKGROUND: Tuberculous pericardial effusion is most often due to the spread of tuberculosis from the mediastinal lymph glands; however, no attempt has yet been made to study these glands. We studied the mediastinal glands in proven tuberculous pericardial effusion patients and hypothesized that the findings may be of use in the etiological diagnosis of pericardial effusion. METHODS AND RESULTS: We studied 45 patients with large pericardial effusion or tamponade. All underwent chest computed tomographic studies that were reviewed by radiologists blinded to the diagnosis. Of these 45 patients, 27 had tuberculosis and 18 had viral or idiopathic effusion. Pericardial biopsy was done in 25/27 and tuberculin skin test in 22/27 patients with tuberculosis, and all received specific treatment. In patients with tuberculosis the skin test measured 17+/-3.3 mm. All 27 had mediastinal lymph glands > or = 10 mm in size. The mean size of the mediastinal glands was 19.5+/-8.6 mm and the mean number was 2.5+/-1.2. The aortopulmonary glands were the most frequently enlarged (63%), and hilar the least often (14.8%). The glands showed a hypodense center in 52% of the patients. On follow-up of 15.8+/-10.4 months, glands were not seen in 80.9%, and were smaller in size in 19%; none had a hypodense center. Marked lymphadenopathy was not seen in any patient with viral/idiopathic pericardial effusion. Two had glands < or = 5 mm in size. CONCLUSIONS: Only patients with tuberculosis had substantial mediastinal lymph gland enlargement and not those with viral or idiopathic pericardial effusion. Such glands disappeared or regressed on treatment. In the appropriate clinical context, marked nonhilar mediastinal lymphadenopathy on chest computed tomographic studies along with a strongly positive tuberculin skin test could be of value in the noninvasive diagnosis of pericardial effusion due to tuberculosis.