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.

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.

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.

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.

Suture repair of pectus excavatum at the time of cardiac surgery on an infant.

An 11-month-old girl was diagnosed with pulmonary atresia with intact ventricular septum and symmetrical pectus excavatum that had developed after prior palliative operation. We performed a transannular patch repair and atrial septal defect closure. Simultaneously, to prevent postoperative right ventricular outflow tract compression, the sternum was elevated by two 1-0 braided polyester horizontal mattress sutures on the posterior side of the third and fourth costal cartilages. Postoperative respiratory distress did not occur and her hemodynamics was stable. Four months later, she is alive and well without recurrence of the thoracic deformity.

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.

Congenital pulmonary vein stenosis with anomalous pulmonary venous connection.

An 11-month-old boy with congenital pulmonary vein stenosis, partial anomalous pulmonary venous connection, and ventricular septal defect is described. Angiocardiography demonstrated stenosis between the right upper pulmonary vein and high superior vena cava and obstruction of the right lower pulmonary vein. For pulmonary vein stenosis, we performed transverse sutured plasty for the right upper pulmonary vein, followed by right lower lobectomy. In some patients, combined management for pulmonary vein stenosis is effective.

Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia.

A 6-month-old boy was diagnosed with coronary sinus orifice atresia, double-outlet right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and moderate common atrioventricular valve regurgitation associated with heterotaxy syndrome. Cardiac venous flow drained through a persistent left superior vena cava. We decided to perform coronary sinus orifice unroofing through the right atrium under a guide using a bougie. The persistent left superior vena cava was divided. Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event.

Palliative management of a low-birth-weight infant with congenitally corrected transposition of the great arteries, severe restrictive foramen ovale, hypoplasia of the morphologically right ventricle, ventricular septal defect, and steno-insufficiency of the tricuspid valve.

A 3-month-old girl weighing 2160 g was diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, severe restrictive foramen ovale, hypoplasia of the morphologically right ventricle, and steno-insufficiency of the tricuspid valve. As her hemodynamic condition (which was comparable to that of the single ventricle with obstructed pulmonary venous pathway) deteriorated progressively, she underwent a palliative operation at 4 months of age. Postoperatively, she was managed with the "low resistance strategy" to achieve a stabilized state of the pulmonary circulation. Currently, 1.5 years after the operation, she is doing remarkably well.

Relationship between increased blood pressure and hematocrit during modified ultrafiltration for pediatric open heart surgery.

OBJECTIVE: Modified ultrafiltration increases blood pressure after cardiopulmonary bypass in children. To investigate the cause of this hemodynamic improvement, we assessed the relationship between increased blood pressure and hematocrit. METHODS: We retrospectively assessed 30 consecutive patients who underwent ventricular septal defect closure, and divided them into two groups: group M (modified ultrafiltration, n=15) and group C (conventional ultra-filtration, n=15). We compared the intraoperative transitions of blood pressure and hematocrit, and analyzed the correlations between blood pressure and hematocrit at 15 min after cardiopulmonary bypass (immediately after modified ultrafiltration in group M) and between the percent increases in blood pressure and hematocrit during modified ultrafiltration. RESULTS: Although intraoperative central venous pressure and dopamine dosage were similar, in group M, increases in hematocrit (26.4% +/- 4.9% to 31.9% +/- 5.7%, P < 0.01) and systolic blood pressure (61.1 +/- 10.3 to 75.6 +/- 11.5 mmHg, P < 0.01) occurred during modified ultrafiltration. Furthermore, diastolic and mean blood pressure at 15 min after cardiopulmonary bypass (after modified ultrafiltration) were higher in group M than in group C. However, systolic, mean, and diastolic blood pressure were not correlated with increased hematocrit after modified ultrafiltration, and there was also no correlation between the percent increases in each blood pressure and hematocrit. CONCLUSION: Modified ultrafiltration increased blood pressure and hematocrit immediately after cardiopulmonary bypass in children. However, no correlations were detected between the increases in blood pressure and hematocrit. These results indicate hemoconcentration is not the major cause of the increased blood pressure during modified ultrafiltration.

Truncus arteriosus repair after palliative bilateral pulmonary artery banding.

A 3.5-month-old girl was diagnosed with type I truncus arteriosus and severe pulmonary hypertension. We performed palliative bilateral pulmonary artery banding (right and left circumferences, 14 and 12 mm, respectively). Corrective repair for truncus arteriosus was then performed at 10 months of age. The good postoperative course in this patient demonstrates that palliative bilateral pulmonary artery banding is a useful surgical option for truncus arteriosus associated with pulmonary hypertension.

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.