Shin Takabayashi

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. Conclusion— 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.

Pediatric Cardiac Remodeling After Cardiac Resynchronization Therapy

A 1.8-year-old male required a conventional DDD pacemaker for an atrioventricular block after congenital heart surgery. Five years later, heart failure due to left ventricular (LV) dyssynchrony progressed and we performed cardiac resynchronization therapy (CRT). Long-term echocardiographic follow-up showed that LV shortening fraction had improved within the first year after CRT, and LV end diastolic dimension had decreased after the first year. During LV remodeling (1–24 months after CRT), the QRS duration shortened without a change in the JT and Tpeak-end interval. The New York Heart Association class improved from III to I during the 2.3-year follow-up.

Fatal Pulmonary Lymphangiectasia Manifesting After Repeated Surgeries for Intractable Chylopericardium and Chylothorax in a 20-Month-Old Girl

A 20-month-old girl was admitted to our hospital because of progressive dyspnea on exertion and a 10-month history of failure to thrive. She had bilateral hearing impairment. The family history was unremarkable. The patient had a hemangioma on the temporal portion of the head and no edema on the legs or face. A chest radiograph (Figure 1A) showed enlargement of the cardiac silhouette with pulmonary congestion. Cardiac ultrasound and computed tomographic scanning of the chest (Figure 1B) revealed copious pericardial effusion with no structural heart diseases. Diagnostic pericardiocentesis produced 150 mL of milky fluid (Figure 1C), which confirmed a diagnosis of chylopericardium because of positive Sudan III staining, a high triglyceride concentration, and a predominance of lymphocytes (>95%). Despite a medium-chain triglyceride-enriched diet and total parenteral nutrition for more than 1 month after pericardiocentesis, the massive pericardial effusion persisted, which prompted surgical management including the clipping of the thoracic duct and creation of a left pericardial …

Strategy for pulmonary atresia and intact ventricular septum

Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.

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.

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.

Dynamic Right Ventricular Outflow Tract Obstruction Due to a Swinging Echolucent Cyst on Interventricular Septum in a 4-Year-Old Girl

A 4-year-old girl was referred to our hospital for evaluation of an incidentally found heart murmur. She had a history of perimembranous ventricular septal defect in infancy, which was reported to be spontaneously closed sonographically in a nearby hospital at 1 year of age. She had no history of traumatic injury. On examination, a grade 4/6 systolic ejection murmur was audible at the left upper sternal border. Chest radiograph and ECG were unremarkable. Echocardiography (Figure 1; Movies I and II in the online-only Data Supplement) revealed that an echolucent cyst (10 mm in diameter) originating from the membranous interventricular septum (IVS) was swinging like a pendulum and obstructing the right ventricular outflow tract, as represented by a peak flow velocity of 4.0 m/s. There was no visible shunt jet across the IVS or the cystic wall. Cardiac catheterization and angiography (Figure 2; Movies III …

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.

Persistent lung shadow in an infant with ventricular septal defect and partial anomalous pulmonary venous connection associated with pulmonary venous obstruction

Preoperative diagnosis of this combination is sometimes challenging: previous cases are reported in the postoperative or postmortem studies. We here report on an infant with a rare association of congenital PVO, ventricular septal defect (VSD), and partial anomalous pulmonary venous connection (PAPVC), which was diagnosed preoperatively in the work-up of failure to thrive and persistent infi ltrated lung shadow.

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.