In vivo plaque composition and morphology in coronary artery lesions in adolescents and young adults long after Kawasaki disease: a virtual histology-intravascular ultrasound study.

BACKGROUND: Coronary artery lesions (CALs) late after Kawasaki disease were characterized by endothelial dysfunction and low-grade inflammation, surrogate markers for atherosclerosis. We tested the hypothesis that CALs in patients long after Kawasaki disease are accompanied by atheroma-like features, as assessed by virtual histology-intravascular ultrasound, a new method to assess coronary plaque composition and morphology in vivo. METHODS AND RESULTS: Virtual histology-intravascular ultrasound was performed in 13 Japanese Kawasaki disease patients (median age, 18.3 years; interquartile range, 16.9 to 23.3 years) an interval after Kawasaki disease (median, 15.9 years; interquartile range, 14.3 to 21.9 years). We investigated 6 sites with localized stenosis, 15 sites with an aneurysm, 29 sites with a regressed aneurysm, and 50 sites with a normal coronary segment. Plaque components were categorized into 4 parts: fibrous, fibrofatty, necrotic core, and dense calcium areas. Qualitatively, the normal segment had no or trivial intravascular ultrasound-visible plaque area, whereas the CAL exhibited a heterogeneous plaque area with the 4 components in different amounts and proportions. Quantitatively, a combined group of CALs had a higher absolute value of fibrous, dense calcium, and necrotic core areas than the normal segment. In further analyses of 3 subtypes of CALs, localized stenosis, an advanced lesion, exhibited higher absolute and relative values of dense calcium and necrotic core areas and a lower relative value of the fibrous area than regressed and persistent aneurysms. CONCLUSIONS: The present limited but initial virtual histology-intravascular ultrasound findings give new insight into the potential role of atherogenesis in the evolution of CALs in adolescents and young adults long after Kawasaki disease and therefore warrant further investigation.

Postnatal course of hypoplastic left heart complex and restrictive foramen ovale.

Hypoplastic left heart complex is a cardiac malformation at the mildest end of the spectrum of hypoplastic left heart syndrome. Recently, biventricular repair was proposed for such patients without retrograde flow to the ascending aorta. However, the preoperative course of these patients is unclear. This report describes a unique case of this complex with a retrograde flow to the ascending aorta and a restrictive foramen ovale at birth in which the patient ultimately underwent biventricular repair after transient pulmonary congestion and full reversal of aortic and ductal flow.

First-stage angioplasty for a single ventricle with pulmonary artery coarctation.

We report first-stage palliation consisting of pulmonary artery angioplasty and a systemic-to-pulmonary shunt using a minimized cardiopulmonary bypass technique performed with modified ultrafiltration for two patients with a single ventricle, pulmonary atresia, patent ductus arteriosus, and pulmonary coarctation during early infancy. Postoperative early extubation (15 and 18 h) and bilateral pulmonary artery growth before the second stage (Nakata indexes, in mm2/m2: right 94, left 53; and right 209, left 70) were experienced. Less-invasive pulmonary artery continuity reconstruction at an early stage is advantageous for low-weight patients with a single ventricle and pulmonary coarctation.

Comparison of hemodynamics between Norwood procedure and systemic-to-pulmonary artery shunt for single right ventricle patients.

OBJECTIVE: Despite that surgical outcomes of patients with hypoplastic left heart syndrome have improved, one of the problems remaining is the high interstage mortality after a stage I Norwood procedure. The purpose of this study was to determine the hemodynamic characteristics of hypoplastic left heart syndrome after a Norwood procedure. We examined the perioperative hemodynamic differences of the staged operation between the first stage of the Norwood procedure and systemic pulmonary shunt for single right ventricle patients. METHODS: Data from 39 patients who underwent a Norwood procedure (right ventricle to pulmonary artery conduit: 19, Blalock-Taussig shunt, 20) were analyzed. There were nine early and seven interstage deaths. Bidirectional cavopulmonary shunt was performed in 15 patients and the Fontan procedure in 9 (group H). We defined the control group as 26 patients who underwent the first stage of a systemic pulmonary shunt for a single ventricle. Bidirectional cavopulmonary shunt was performed in 14 patients and the Fontan procedure in 8 (group C). We compared the perioperative hemodynamics of the staged operation between the two groups. RESULTS: Cardiothoracic ratio and single ventricular diastolic dimension before bidirectional cavopulmonary shunt were acutely increased in group H (P=0.02, <0.001). There was no significant difference between the two different types of Norwood procedures. The pulmonary artery index for the right heart bypass operation was lower in group H than in group C (P<0.001). Oxygen saturation before bidirectional cavopulmonary shunt in group H decreased (P<0.001) and thus was lower than that in group C (P=0.003). Mortality and the postoperative clinical parameters of the right heart bypass operation were not different between the two groups. CONCLUSIONS: Patients with hypoplastic left heart syndrome showed hemodynamic instability of acutely increased cardiothoracic ratio, and single ventricular diastolic dimension despite decreased oxygen saturation interstage after stage I of a Norwood procedure. This suggests that this hemodynamic characteristics in hypoplastic left heart syndrome correlates with the higher mortality before second stage palliation than in found with single right ventricle patients.

Ross procedure for congenital aortic insufficiency and an associated right intramural coronary artery.

A 7-year-old boy diagnosed with aortic insufficiency was treated with a Ross procedure and he had an associated right intramural coronary artery. Although preoperative angiocardiography showed that the right and left coronary artery orifices were close together, a right intramural coronary artery could not be diagnosed by transthoracic echocardiography. Intraoperative findings showed that the right coronary artery was intramural for a distance of 7 mm. As a result, a longer single coronary button was harvested and transplanted to the pulmonary autograft. The postoperative course was uneventful; coronary ischemia did not occur and aortic valve function was preserved.

Emergent permanent pacemaker implantation in a premature 1,502 g neonate.

We report a case of an emergent pacemaker implantation in a 1,502 g preterm neonate immediately after birth due to congenital complete atrioventricular block. At a gestational age of 29 weeks the patient was delivered by cesarean section followed by unsuccessful drug treatment of the atrioventricular block. Sixty-five minutes after birth the patient underwent permanent pacemaker implantation. Through a subxyphoid approach, a lead was fixed to the epicardium of the right ventricle, and connected to a pulse generator inserted between the rectus abdominus muscle and posterior rectus sheath. The patient is alive and well 16 months after the operation without pacemaker failure.

Surgical closure of an apical ventricular septal defect through a septal ventriculotomy.

Here we report a 3-year and 7-month-old boy with a muscular ventricular septal defect at the apex. At age 7 months, pulmonary artery banding was performed. Surgical treatment consisted of double patch closure via septal ventriculotomy and a main pulmonary artery plasty by an end-to-end anastomosis with cardiopulmonary bypass. There was no residual leak of the ventricular septal defect or ventricular aneurysm and the postoperative course was good. This technique could be a useful surgical option for repair of apical muscular ventricular septal defects.

Successful surgical treatment for an adult case of double aortic arch.

We present the case of a 39-year-old male who had complaints of dysphagia and throat pain. Computed tomography (CT), 3 dimensional CT and aortography revealed a double aortic arch (Edwards type IA). The patient underwent exploration through a left-sided thoracotomy and the left arch was divided at the distal site of the left subclavian artery, which completely relieved the esophageal compression.

Postoperative prone position management of tetralogy of fallot with absent pulmonary valve syndrome.

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with respiratory failure both before and after surgery. This report describes our experience using prone positioning with bilateral pillows to avoid compression of the anterior chest wall after surgery. In the case here, the patient's respiratory distress was improved by this positioning. Prone position and avoiding anterior chest compression has an effect on severe respiratory distress of tetralogy of Fallot with absent pulmonary valve syndrome.

Performance of the Fontan procedure using extra-cardiac direct anastomosis on patients treated for penicillin-resistant Streptococcus pneumoniae endocarditis.

Streptococcus pneumoniae is now a rare cause of endocarditis in humans. We report a patient with a double outlet right ventricle and mitral atresia, who underwent the Fontan procedure without prosthetic materials after treatment for penicillin-resistant Streptococcus pneumoniae endocarditis. Postoperative infectious endocarditis was not found. In patients with a history of infectious endocarditis, direct anastomosis of the main pulmonary artery and inferior vena cava would reduce the risk of recurrent infectious endocarditis.

Modified Ross procedure using a conduit with a synthetic valve.

OBJECTIVE: In the Ross procedure, a homograft conduit is commonly used in place of an autotransplanted pulmonary valve. Homograft availability may be a problem and has resulted in a search for alternatives. We performed a modified Ross procedure for right ventricular outflow tract reconstruction with a synthetic valved conduit as an alternative to homograft. Our early results of valvular and right ventricular function were evaluated in patients who used a conduit with a synthetic valve. METHODS: Subjects consisted of 11 patients, who ranged in age from 5 to 22 years (12.0+/-4.9), and whose body weight ranged from 15.1 to 52.5 (34.3+/-14.4) kg. Indications for surgery were aortic stenosis (n=3), aortic stenosis and regurgitation (n=4), and aortic regurgitation (n=4). Right ventricular outflow tract reconstruction was performed using a hand-fashioned valved conduit prepared by sewing a 0.1 mm thick polytetrafluoroethylene sheet onto the luminal cavity of the 20-28 mm conduit. A conduit made with polytetrafluoroethylene was used in 8 patients, and a Dacron graft was used in 3 patients. RESULTS: There was no in-hospital or late mortality and angiocardiography at discharge revealed that all artificial valves remained active. The mean right atrial pressure and right ventricular end-diastolic pressure were not statistically different from preoperative values. The latest echocardiography (mean interval, 12.6 months) revealed that a mean pressure gradient across the synthetic valve was 11.4+/-11.1 mmHg and none of the patients had moderate or severe regurgitation. CONCLUSIONS: We demonstrated that a modified Ross procedure for right ventricular outflow tract reconstruction using a conduit with an appropriate synthetic valve is particularly effective in older children.

Spontaneous closure of ductus arteriosus in interrupted aortic arch with ventricular septal defect.

A 2-month-old boy diagnosed with interrupted aortic arch type B was treated with a two-stage procedure. His ductus arteriosus had closed spontaneously. Collaterals via both vertebral arteries developed. A 15-mm stenotic segment existed between the left subclavian artery and the descending aorta. The direct anastomosis between the common carotid artery and the descending aorta was performed as a first palliation at the age of 3 months. The left subclavian artery was reconstructed by end-to-side anastomosis to the descending aorta. The postoperative course was uneventful. The closure of ventricular septal defect and pulmonary artery debanding were performed as a second operation 4 months after the first palliation. The patient is alive and well 7 months after the second operation.

Congenital bilateral diaphragmatic eventration with membranous chest wall hamartoma.

The case of a 2-month-old girl with congenital bilateral eventration of the diaphragm is reported. After normal delivery from cephalic position, the patient developed respiratory distress. Mechanical ventilation started immediately but extubation was difficult. A membranous lesion was found on computed tomography of the chest. Resection of the membrane between the right middle and lower lobes and bilateral diaphragmatic plication was performed. Histologically the membrane was a chest wall hamartoma. The patient was extubated on 6 day postoperatively and is alive and well 4 months after surgery.

Patch angioplasty and neo-ostium creation for intramural left coronary artery.

Anomalous aortic origin of the coronary artery is a rare cardiac anomaly which induces myocardial ischemia and is associated with sudden death. We operated on a 25-year-old female with syncopal episodes who had an intramural left coronary artery. A neo-ostium was created in the left sinus but the initial neo-ostium seemed small because of the hypoplastic intramural segment of the left coronary artery. Therefore, saphenous vein patch angioplasty was added for ostial enlargement. The patient was symptom-free at one year follow-up and exercise stress test was negative for ischemia.

Removal of prostaglandin E2 and increased intraoperative blood pressure during modified ultrafiltration in pediatric cardiac surgery.

OBJECTIVE: We investigated the relationship between serum prostaglandin E(2) and intraoperative blood pressure in pediatric cardiac surgery with modified ultrafiltration. METHODS: In 35 consecutive patients (31.6 +/- 26.8 months, 0.4-111 months, 10.9 +/- 5.5 kg, 2.9-23.8 kg) who underwent cardiac surgery with modified ultrafiltration, we measured intraoperative serum prostaglandin E(2) changes and effluent prostaglandin E(2), assessed the relationship between serum prostaglandin E(2) and intraoperative hemodynamic parameters, and performed subset analyses to compare patients with low (<10 kg, n = 18) and high (>10 kg, n = 10) weights. RESULTS: During cardiopulmonary bypass, systolic blood pressure decreased from 80.8 +/- 15.2 to 60.5 +/- 11.3 mm Hg (P = .00000002979) and serum prostaglandin E(2) increased from 16.6 +/- 8.7 to 58.8 +/- 53.3 pg/mL (P = .002). During modified ultrafiltration, although central venous pressure and catecholamine dosage transited at the same levels, systolic blood pressure increased from 60.5 +/- 11.3 to 83.4 +/- 14.1 mm Hg (P = .00000002979) and serum prostaglandin E(2) decreased from 58.8 +/- 53.3 to 21.1 +/- 11.6 pg/mL (P = .001), with negative correlation between serum prostaglandin E(2) and systolic blood pressure (R = -0.392, P = .0000277723) and 15,700 +/- 10,700 pg (1790 +/- 2230 pg/kg) prostaglandin E(2) removed during modified ultrafiltration. Decrease in serum prostaglandin E(2) was significantly higher in low-weight patients (51.8 +/- 58.4 pg/mL) than in high-weight patients (15.7 +/- 30.1 pg/mL). CONCLUSION: Removal of prostaglandin E(2) is one reason for increased blood pressure during modified ultrafiltration, with the effect more marked in low-weight patients.

Pulmonary artery growth after Norwood and bidirectional Glenn procedure.

A 19-day-old boy diagnosed with hypoplastic left heart syndrome underwent stage I bilateral pulmonary artery banding and main pulmonary artery-to-descending aorta shunt. A restrictive atrial septal defect existing before stage I recurred after balloon atrioseptostomy. After stage II Norwood and bidirectional Glenn procedure at age nine months, the Nakata index decreased to 73 mm(2)/m(2) (pulmonary artery mean pressure: 15 mmHg) and multiple systemic venous collaterals developed. Thus, we instituted oral sildenafil medication, and undertook surgical chest subcutaneous venous ligation and coil embolizations. Three years later, the Nakata index had increased to 117 mm(2)/m(2) (pulmonary artery mean pressure: 13 mmHg) and a Fontan procedure was successfully performed.