Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis / 日本心臓血管外科学会雑誌

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.

Physiological Changes and Clinical Implications of Syndromic Craniosynostosis

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.

Treatment and Outcome of Acute Aortic Dissection Associated with Atherosclerotic Aortic Aneurysm / 日本心臓血管外科学会雑誌

We set out to assess our treatment strategy of acute aortic dissection associated with atherosclerotic aortic aneurysm, and to assess its results. A total of 228 patients with acute aortic dissection were admitted to our hospital between 1994 and 2009. Among these, 30 cases were associated with atherosclerotic aortic aneurysm and we retrospectively analyzed their patient data. Of these, 5 patients received diagnoses of Stanford A dissection and 25 patients demonstrated Stanford B. Coexisting aneurysms consisted of postabdominal aortic replacement in 9 patients, ascending aortic aneurysm in 1, arch aortic aneurysm in 6, descending aortic aneurysm in 2, thoracoabdominal aortic aneurysm in 3, and abdominal aortic aneurysm in 9. Patients were divided into 3 groups based on the relationship between aneurysm and dissection : acute aortic dissection occurred after graft replacement of an aortic aneurysm (Group 1, <i>n</i>=9), dissection coexisted with aneurysm in a different segment of the aorta (Group 2, <i>n</i>=8), and dissection extended to or involved the aneurysm (Group 3, <i>n</i>=13). Our treatment strategy for all patients excluding those with aortic rupture or malperfusion is described below. In the cases of Stanford A dissection, emergency ascending aortic replacement or total arch replacement was performed. In cases of Stanford B, patients were treated conservatively in the acute phase. Surgery for the coexisting aortic aneurysm was then performed in the chronic phase, if the aneurysm was large. We think that the interval between dissection onset and aneurysm surgery should be extended to at least 1 month, because the aortic wall was too fragile to perform anastomosis in the acute phase in the present cases. As a result, there were 2 hospital deaths in Group 3, but there was no aortic rupture during treatment in the acute phase in any of these 3 groups. There were no vascular-related deaths during follow up. Our treatment strategy obtained favorable outcomes.