Biventricular Repair Conversion After Temporary One and a Half Ventricular Repair for Hypoplastic Right Ventricle.

This case was an infant with critical pulmonary stenosis at birth. A temporary one and a half ventricular repair was performed to increase right ventricular end-diastolic volume and the size of the tricuspid valve annulus before biventricular repair conversion. The one and a half ventricular repair was performed using a unidirectional bicaval Glenn anastomosis. The right ventricular end-diastolic volume at 3 years was 73.2% of normal value but with a 64/36 right/left lung perfusion ratio. An anatomic biventricular correction included removal of the bicaval Glenn shunt and reconstruction of the continuity between the right and main pulmonary arteries. The one and a half ventricular repair made the reconstruction feasible.

A Successful Case of External Stenting for Bronchomalacia Lasting Over 20 Years.

We report on a patient with perimembranous ventricular septal defect and pulmonary hypertension. He underwent pulmonary artery banding followed by external stenting of the left bronchus because of secondary bronchomalacia. The external stent applied was a ringed expanded polytetrafluoroethylene graft, 10 mm in diameter and fixed at 8 points. Intracardiac repair was performed at the age of 2 years and the external stent was left in place. Since then, the patient has been healthy and computed tomography reveals that the external stent graft has retained function for over 20 years.

External stenting: A reliable technique to relieve airway obstruction in small children.

OBJECTIVE: Airway obstruction in children may be caused by conditions such as vascular compression and congenital tracheobronchomalacia. Obstructive pulmonary vascular disease may be a detrimental sequel for patients with congenital heart disease. We evaluate our own original external stenting technique as a treatment option for these patients. METHODS: Ninety-eight patients underwent external stenting (1997-2015). Cardiovascular anomalies were noted in 82 (83.7%). Nine patients had hypoplastic left heart syndrome and 6 had other types of single-ventricular hearts. RESULTS: The median age at the first operation was 7.2 months (range, 1.0-77.1 months). The mechanisms were tracheobronchomalacia with (n = 46) or without (n = 52) vascular compression. Patients underwent 127 external stentings for 139 obstruction sites (62 trachea, 55 left bronchus, and 22 right bronchus). The stent sizes varied from 12 to 16 mm. There were 14 (8 in the hospital and 6 after discharge) mortality cases. Nine required reoperation for restenosis and 3 required stent removal for infection. The actuarial freedom from mortality and any kind of reoperation was 74.7% ± 4.6% after 2.8 years. The negative pressure threshold to induce airway collapse for congenital malacia (n = 58) improved from -15.9 to -116.0 cmH2O. A follow-up computed tomography scan (>2.0 years interval from the operation; n = 23) showed the mean diameter of the stented segment at 88.5% ± 13.7% (bronchus) and 94.5% ± 8.2% (trachea) of the reference. CONCLUSIONS: External stenting is a reliable method to relieve airway compression for small children, allowing an age-proportional growth of the airway.

Tracheoplasty using the thymus against tracheo-esophageal fistula due to necrotizing tracheobronchitis in a very low birth weight infant.

We report a case of a very low birth weight infant treated successfully with tracheoplasty using the thymus against tracheo-esophageal fistula due to necrotizing tracheobronchitis. A baby boy was born at 31 weeks gestation with a birth body weight of 1,230 g. Suddenly on Day 19, his respiration deteriorated, and a flexible bronchofiberscopy showed tracheostenosis, tracheomalacia, and tracheal diverticula. On Day 21, his abdomen became significantly distended, and a flexible bronchofiberscopy showed a tracheo-esophageal fistula due to a rupture of the diverticula. On Day 105, during surgery, we confirmed necrosis of the trachea. Because the lesion was widespread, we used the thymus for tracheoplasty and closure of the tracheo-esophageal fistula. His post-operative course went well, and was discharged at 1 year 9 months of age. Now, 8 years after the operation, although he still needs a T-tube airway management through tracheostomy, he has achieved speech, normal growth and development, and takes regular classes at school. Tracheoplasty using the thymus is thought to be a treatment worth considering when there are no other effective alternative treatments for tracheo-esophageal fistula due to necrotizing tracheobronchitis.

External stabilization for severe tracheobronchomalacia using separated ring-reinforced ePTFE grafts is effective and safe on a long-term basis.

PURPOSE: Tracheobronchomalacia is a respiratory disorder that results from abnormal cartilage development. Since 2003, we have performed external stabilization for severe tracheobronchomalacia using separated ring-reinforced expanded polytetrafluoroethylene (ePTFE) grafts. The aim of this study was to analyze the short- and long-term outcomes of our procedure. METHODS: We reviewed the charts of patients who underwent external tracheobronchial stabilization from January 2003 through December 2012. Separated ring-reinforced ePTFE grafts were fixed to the anterior and posterior lesions of malacia independently, with confirmation of a well-opened lumen by bronchoscopy. RESULTS: Five children with severe tracheobronchomalacia underwent six operations. The median follow-up period was 43 months (range 11-109 months). There was no operative mortality associated with the procedure. One patient required removal of a section of the graft owing to the development of bronchial granulation tissue at the site of fixation. All patients showed improved respiratory function, and tracheal growth was confirmed by follow-up computed tomography. CONCLUSIONS: External stabilization with separated ring-reinforced ePTFE grafts for severe tracheobronchomalacia is effective, less invasive than alternatives and preserves the growth potential of the affected airway segment.

Removal of an external stent of the bronchus.

We report a case of a patient who underwent removal of an external stent of the bronchus. She had undergone external stenting for tracheobronchomalacia. After removal of the external stent, the bronchus was just as widely patent as preoperative state.

Left ventricular-right atrial communication complicated by aortic regurgitation.

We report a case of left ventricular-right atrial communication complicated by aortic valve incompetence in a 29-year-old man. The patient had a history of heart murmur during childhood. There were no clinical signs of infection. We performed plication of the aortic valve and patch closure of the left ventricular-right atrial communication under cardiopulmonary bypass. The patient improved immediately after the operation.

Successful extracardiac total cavo pulmonary connection (TCPC) after external tracheobronchial stenting for tracheobronchomalacia.

OBJECTIVES: A girl who was diagnosed with cyanotic congenital heart disease in a newborn, showed anoxic spell attacks which we thought were correlated with the congenital heart disease. Although she underwent the palliative operation at 8 months old and her SpO2 was increased, she experienced severe and life-threatening respiratory dysfunction many times after that. After careful examinations, the respiratory complaint was proved to be not only due to cyanotic congenital heart disease but also tracheobronchomalacia. METHOD: She had undergone the external stenting to the trachea and right bronchus at 1 year old. After that, she was examined by cardiac catheterization and the Fontan-type operation was successful using a tissue-engineered graft at 2 years old. RESULTS: Her post-operative course was uneventful and she was discharged. CONCLUSION: It is very important to remember the possible existence of tracheobronchomalacia and prevent a life-threatening attack when congenital cardiac patients experience a prolonged respiratory failure or abnormal respiration.

Complete atrioventricular canal and tetralogy of Fallot with pulmonary atresia.

Our patient was diagnosed with complete atrioventricular canal and Tetralogy of Fallot with pulmonary atresia at the age of 1 month. Then he underwent right and left Blalock-Taussig shunts at the ages of 2 months and 5 years, respectively. His cyanosis had increased at 20 years of age. Cardiac catheterization showed occlusion of the left Blalock-Taussig shunt and absence of the left pulmonary artery. Lung perfusion scintigram showed late phase perfusion in the left lung. Chest computed tomographic scan demonstrated the left pulmonary artery. We describe the operative technique of total correction.

Quantitative assessment of tracheal collapsibility in infants with tracheomalacia.

Infantile tracheomalacia is a potentially life-threatening disease requiring prolonged artificial respiratory support. Diagnosis and management of this disease may be further improved by establishing a suitable objective and quantitative assessment protocol for tracheal collapsibility. It is our hypothesis that tracheal collapsibility can be represented by the relationship between intraluminal pressure and the cross-sectional area of the trachea. To test this hypothesis, static pressure/area relationships of the trachea were obtained from anesthetized and paralyzed infants, who were diagnosed as having tracheomalacia by endoscopic observation. These relationships were fitted on a linear regression model, followed by calculation of the estimated closing pressure. The tracheal closing pressure ranged from -8 to -27 cm H(2)O, suggesting easy collapsibility of the trachea during crying or coughing and noncollapsibility during the spontaneous respiratory cycle, which coincided with the infants' symptoms. It is our conclusion that tracheal collapsibility of infants with tracheomalacia can be quantitatively assessed by the static pressure/area relationship of the trachea obtained under general anesthesia and paralysis.

Simultaneous repair of pectus excavatum and congenital heart defect in adults by using the convex bar.

Simultaneous repair of pectus excavatum and cardiac lesions remains technically difficult. In adults, most repairs of pectus deformity and heart lesions have been performed through long incisions, sternal splits, excision of deformed cartilages, and sternal turnover, which can result in poor cosmetic appearance because of sternal devascularization. We performed concomitant repair of pectus excavatum and an atrial septal defect through a short midline incision in an adult. The sternum was fixed by using absorbable plates and screws and was supported by a convex steel bar. The cosmetic appearance remained excellent after the operation. The technique and a review of the literature are included.