Tomonori Higuma

Ventricular septal dysfunction after surgical closure of multiple ventricular septal defects.

BACKGROUND We assessed the global and regional ventricular septal functions using conventional echocardiography and two-dimensional speckle tracking imaging in children with postoperative multiple ventricular septal defects. METHODS Thirty-six children were studied: 16 with postoperative multiple ventricular septal defects and 20 normal control subjects. In children with multiple ventricular septal defects, 60 ventricular septal defects were closed using one of three different techniques (patch closure, the sandwich technique, direct closure). Speckle tracking imaging was applied to three short-axis echocardiographic images. RESULTS The total patch area used in the multiple ventricular septal defects group was correlated with the postoperative ejection fraction (r=0.703) and Tei index (r=0.778). The global septal peak systolic radial displacement and global septal peak systolic radial strain in the multiple ventricular septal defects group were significantly lower than those observed in the control subjects. The peak systolic radial strain in the segments closed with patches and the peak systolic radial displacement in the segments closed with the felt sandwich technique were significantly lower than those observed in the intact septal segments. No significant regional functional depressions were identified in the segments that were closed directly. CONCLUSIONS The postoperative ventricular global and septal functions were significantly reduced in children with multiple ventricular septal defects, especially in the cases with complex congenital heart disease and that were closed with large prosthetic materials. These results suggest that an effort to minimize the use of patch materials may lead to preserved postoperative ventricular function.

Congenital sternal cleft.

We describe a rare case of congenital sternal cleft. Multidetector computed tomography showed the counterclockwise rotation of the heart. Primary surgical correction was successfully performed using the Sabiston technique when the patient was 20 days old. Although the circulatory and respiratory status was unstable in the immediate postoperative period, this abated, and the patient was discharged without complications on the 13th postoperative day. Postoperative multidetector computed tomography showed that the heart approached the normal position.

Management of pulmonary venous obstruction

Although improved surgical techniques have led to significantly better outcomes of surgery for total anomalous pulmonary venous connection, the risk of progressive pulmonary venous obstruction continues to be a clinical problem. Both obstructed total anomalous pulmonary venous connection and post-repair pulmonary venous obstruction are associated with a significant risk of recurrent obstruction or death, requiring reoperation for stenosis. In general, side to side anastomosis of the pulmonary venous confluence to the functional left atrium has been performed for supracardiac and infracardiac total anomalous pulmonary venous connection. Repair of total anomalous pulmonary venous connection to the coronary sinus invariably involved unroofing the coronary sinus, followed by pericardial patch closure of the atrial septal defect. Recently, sutureless technique has been adopted as the primary operation for the subgroups of patients that are thought to be at high risk for post-repair pulmonary venous obstruction, such as those with total anomalous pulmonary venous connection associated with right isomerism, infracardiac total anomalous pulmonary venous connection with small individual pulmonary veins, or mixed-type total anomalous pulmonary venous connection. Because the sutureless technique does not require direct anastomosis to the confluence, aggressive resection of the obstructed pulmonary venous tissue can be achieved, and surgically induced distortion of the suture line can be avoided, which may help to prevent subsequent pulmonary venous obstruction. Conventional management strategies for recurrent pulmonary venous obstruction have typically been associated with poor outcomes. Recent reports have supported the use of the sutureless technique to treat post-repair pulmonary venous obstruction.

Anomalous origin of the right coronary artery evaluated with multidetector computed tomography and its clinical relevance

BACKGROUND Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly that may cause myocardial ischemia and sudden death. METHODS We reviewed the clinicopathological records of three cases of AORCA, and compared these with two cases of sudden cardiac death with AORCA revealed by autopsy. RESULTS We report three juvenile cases with an AORCA originating above the commissural junction between the left and right aortic sinuses, with interarterial and intramural compression. They presented with exertional symptoms and were diagnosed with an AORCA by multidetector computed tomography (MDCT), which successfully delineated the spatial resolution of the anomalous origin and course of the right coronary artery (RCA), in the operating room. All three underwent successful surgical unroofing of the RCA. Two cases of sudden cardiac death with AORCA revealed by autopsy showed a slit-like orifice, acute-angled take-off, and long intramural course of the RCA, resembling the RCAs of three juvenile cases. CONCLUSIONS It is crucial to be alert to the presentation of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery, such as those observed in these three cases. MDCT provided an excellent definition and spatial resolution of the unusual origin and intramural course of the RCA, facilitating the correct surgical remedy and resulting in a good outcome for the patients.

Left Atrial Decompression Via Minithoracotomy During Extracorporeal Life Support

Extracorporeal membrane oxygenation through cervical cannulation is an established option for pediatric patients with acute cardiopulmonary failure. However, left-sided heart decompression is sometimes mandatory in patients with severe left ventricular dysfunction. This report describes a fast and less invasive technique for placing a left atrial cannula through a left anterior minithoracotomy approach. In 4 critically ill children, this minimally invasive technique provided satisfactory left-sided heart decompression, and this report describes a representative case.

Endothelin-1 may play an important role in the Fontan circulation

OBJECTIVES Our goal was to evaluate whether endothlin-1 (ET-1) plays an important role in the Fontan circulation. METHODS Thirteen patients with single-ventricle physiology (Glenn circulation, n = 7; Fontan circulation, n = 6) were evaluated using lung histopathological and immunohistochemical studies and then compared with the normal autopsied controls without congenital heart disease (n = 13). We evaluated the medial thickness of the small pulmonary arteries. For 10 of these patients, quantitative real-time polymerase chain reaction analyses of ET-1, endothelin receptors Type A and Type B, endothelin-converting enzyme-1 and endothelial nitric oxide synthase were performed. RESULTS The medial thickness of the small pulmonary arteries in patients with single-ventricle physiology was greater than that of those in the control group (P = 0.0341). Severe medial hypertrophy of the pulmonary arteries was observed in patients who had poor outcomes. Immunohistochemical studies revealed that the marked expression of ET-1 was observed in the endothelium and media of their pulmonary arteries. In these patients, the messenger RNA expression of ET-1 was also increased. Two patients showed high levels of expression of ETAR and ETBR, although these 2 cases maintain good Fontan circulation. CONCLUSIONS Medial hypertrophy and the overexpression of ET-1 in the pulmonary arteries were observed in some patients in whom the Fontan circulation failed. Our data suggest that ET-1 may play an important role in maintaining the Fontan circulation.

Follow-Up of Persistent Tracheal Stenosis After Surgery for a Double Aortic Arch.

Persistent respiratory symptoms often occur after surgical repair of double aortic arch (DAA). Most often, symptoms are relatively mild and tend to be self-limited and improve with growth. Multidetector computed tomography (MDCT) imaging can be used to obtain needed anatomic information regarding the potential for extrinsic airway compression and is minimally invasive, safe, and readily available after surgery. We herein report the cases of two patients with persistent airway symptoms after surgical therapy for a double aortic arch. One of the patients eventually required aortopexy in order to achieve complete relief from recurrent symptoms of airway compression. In the other case, wheezing persisted following surgical division of the vascular ring, but it resolved over time without further intervention. In both cases, serial follow-up imaging with computed tomography scans was informative and helped guide management decisions.

Pseudoaneurysm following percutaneous balloon angioplasty for aortic arch recoarctation after the Norwood procedure

We experienced a rare case of 5-month-old male infant presenting with a pseudoaneurysm following percutaneous balloon angioplasty for aortic arch recoarctation after undergoing the Norwood procedure. The pseudoaneurysm, which measured 1 cm in diameter, was located between the left carotid artery and the left subclavian artery. Under deep hypothermic circulatory arrest, resection of the pseudoaneurysm and reconstruction of the neoaortic arch were performed successfully.