室上嵴上室间隔缺损的外科治疗

本文报道５６例室上嵴上室间隔缺损的外科治疗。男３４例，女２２例，年龄３～３９岁。１９例合并主动脉瓣叶脱垂、主动脉瓣关闭不全或佛氏窦瘤破裂。经右房切口修补室缺２例，右室漏斗切口４７例，肺动脉切９７例。直接修补缺损２６例，补片修补３０例。主动脉瓣成形术１例，主动脉瓣置换术５例。５４例全愈出院，死亡２例。作者认为室上嵴上空间隔缺损应尽早手术，以免并发主动脉瓣损害和佛氏窦瘤破裂；中度主动脉瓣关闭不全可行主动脉瓣成形术，采用肺动脉切口优于右室漏斗部切口。     

Surgical experience with Aneurysm of the Sinus of Valsalva

During a three year period from July 1977 to July 1980, six cases of Aneurysm of the Sinus of Valsalva underwent surgical correction in our Institute. Three of them were males and three females. The constant symptoms were palpitations and fatigability and the constant signs were collapsing pulse and continuous murmur with thrill. In four cases the right coronary sinus had ruptured into the right ventricular outflow; in two patients, the non-coronary sinus was involved, one rupturing into the right atrium, the other one being unruptured. Four patients had associated aortic regurgitation and two had ventricular septal defect. All were corrected surgically; four patients with aortic regurgitation had aortic valve replacement; the ventricular septal defects were closed directly. One patient died on the operating table; one died at home suddenly, two months after surgery. The other four patients are doing well clinically, two to five years after surgery.     

Basilar skull fracture: a risk factor for transverse/sigmoid venous sinus obstruction

In trauma practice, basilar skull fracture is an extremely common finding while transverse/sigmoid venous sinus thrombosis is generally considered quite a rare complication. During evaluation of cervical computed tomography (CT) angiography after trauma, we identified five patients in just three months with unexpected transverse/sigmoid venous sinus obstruction ipsilateral to a basilar skull fracture. This number represented a surprisingly high percentage of our neurosurgical trauma consults for the study period (31%). Three of the five patients were found to have sinus thrombosis: two with right transverse/sigmoid sinus thrombosis experienced significant neurological deficits and prolonged hospital courses even with anti-coagulation therapy; one patient with a left transverse/sigmoid sinus thrombosis had a good outcome with anti-coagulation therapy. The other two of the five patients had outflow obstruction, likely from focal epidural bleeding and extrinsic compression: one patient with partial obstruction in the right transverse-sigmoid junction, due to epidural bleeding, experienced a difficult recovery; one patient with a right sigmoid sinus obstruction presented and remained asymptomatic and experienced a benign hospital course. Two of the five patients had a posterior temporal hemorrhagic area ipsilateral to the affected sinus, suggesting that this finding may have represented hemorrhagic venous infarction rather than traumatic contusion. We propose that a basilar skull fracture in the region of temporal or occipital bone should be considered as a significant risk factor for the development of transverse/sigmoid venous sinus obstruction and may be an under-recognized and treatable cause of increased intracranial pressure. Failure to detect this complication may explain, in part, unexpected clinical outcomes.     

Ruptured aneurysms of the sinus of Valsalva in Oriental patients

Between 1964 and 1987, a total of 57 cases of ruptured aneurysm of the sinus of Valsalva underwent surgical correction at the National Taiwan University Hospital. This represents 0.96% of all cardiac operations. The origin of ruptured aneurysm of the sinus of Valsalva was the right coronary sinus in 46, the noncoronary sinus in nine, and the left coronary sinus in two. The aneurysms ruptured into the right ventricle in 44, into the right atrium in 11, into the left ventricle in one, and into both the right ventricle and right atrium in one. Associated congenital cardiac anomalies included ventricular septal defect in 30 patients, aortic regurgitation in 20, and infundibular pulmonic stenosis and coarctation of the aorta in one each. Operative death occurred in two patients (3.5%) and one patient had a successful reoperation. The remainder did well following surgery. To compare the differences between Oriental and Western countries in ruptured aneurysm of the sinus of Valsalva, 361 cases (195 Oriental patients versus 166 Western) were collected from the literature. Analyses of these cases revealed that ruptured aneurysm of the sinus of Valsalva in Oriental patients compared with Western series is characterized by a higher incidence (5 times), more aneurysms originating from the right coronary sinus (87.9% versus 63.6%), more aneurysm rupturing into the right ventricle (84.2% versus 56.6%), a higher incidence of association with ventricular septal defect (mainly supracristal) (59.0% versus 34.6%), less incidence of association with other congenital cardiac abnormalities (4.1% versus 21.5%), very few instances of rupturing into cardiac chambers other than the right ventricle and right atrium, and less incidence of occurrence in the extremities of ages (the youngest was 7 years in Oriental patients versus 11 months in the Western series). In other words, ruptured aneurysm of the sinus of Valsalva in Oriental patients is more or less a simple and uniform disease entity in contrast to the more diverse and protean pathologic profiles encountered in Western series. However, both Oriental patient and Western patient series have similar incidences of combination with aortic regurgitation (24.6% versus 20.0%), with 40.4% of Oriental patients and 60.6% of Western patients presenting with intact ventricular septum. Therefore the pathogenetic mechanisms of ruptured aneurysm of the sinus of Valsalva may at the same time contribute to the development of aortic regurgitation.     

A case of dural arteriovenous fistula of the inferior petrosal sinus successfully treated by transarterial and transvenous embolizations

A 69-year-old male was admitted with chemosis and exophthalmos of his right eye. Angiograms revealed a dural arteriovenous fistula (AVF) involving the right inferior petrosal sinus. The AVF was fed by the right occipital and ascending pharyngeal arteries and drained into the cavernous sinus and right superior ophthalmic vein from the right inferior petrosal sinus. He was treated by transarterial embolization with polyvinyl alcohol in order to reduce the shunt-flow through the fistula. Then he was treated by transvenous embolization with GDC coils five days after the arterial embolization. Symptoms in his right eye have completely disappeared. Transvenous embolization combined with transarterial embolization is a useful and safe approach in the management of AVF involving the inferior petrosal sinus.     

Repair of complete atrioventricular septal defects: results with maintenance of the coronary sinus on the right atrial side

BACKGROUND: This study was undertaken to determine that maintaining coronary sinus on the right atrial side during the surgical repair of complete atrioventricular septal defect (AVSD) does not increase the risk of postoperative complete heart block. METHODS: This is a retrospective study of 51 consecutive patients who underwent biventricular repair of complete AVSD from September 2000 to January 2005. Electrocardiograms and operative data were analyzed. RESULTS: The mean age was 13.3 months (4 to 60). In all the 51 patients, except 13 cases, repair was performed using the two-patch technique. All atrial septal defects were closed using the patch technique with the coronary sinus maintained on the right atrial side in 48 (94%) cases. The cleft in the neomitral valve was closed in all patients. Associated lesions were repaired in four patients (7.8%); coarctation of aorta in two patients; multiple ventricular septal defects (VSD) with coronary sinus type-total anomalous pulmonary venous drainage and right-sided diaphragmatic eventration in one patient; and tetralogy of Fallot in one patient. There were five deaths (9.8%) in a series. The mean hospital stay was 11.8 days. During the same hospitalization, reintervention was required in two cases: one for residual VSD and the other for a severely dysplastic regurgitant mitral valve. Mean follow-up was 11.3 months. One patient required reoperation for residual VSD, residual atrial septal defect, and moderate mitral regurgitation 5 months after the initial repair. Except for first-degree heart block documented in nine cases and right bundle branch block in two cases, all patients remained in sinus rhythm on follow-up electrocardiography as preoperatively documented. No patient required prolonged cardiac pacing in the postoperative period. CONCLUSION: We believe that maintenance of the coronary sinus on the right side can be safely accomplished in the majority of complete AVSD repair as long as careful attention is paid to the anatomy of the conduction system. This technique did not increase the risk of postoperative heart block and permanent pacemaker insertion was not required.     

Aortic root remodeling in atheromatous aneurysms: the role of selected sinus repair.

OBJECTIVE: Atheromatous ascending aortic aneurysms (AAA) frequently present with aortic regurgitation (AR) from dilatation of the sino-tubular junction (STJ) and extension of the pathological process into the root. Experience suggests that root dilatation begins in the non-coronary, then right coronary sinus. Rather than employ aortic root replacement or the David procedure, we have elected to replace the ascending aorta and remodel the STJ and involved sinuses. We studied the outcome after selective sinus replacement in 29 consecutive AAA patients between 1995 and 2001. METHODS: There were nine male and 20 females. Age ranged from 47 to 79 years (mean 67.5). Seven had arch aneurysms and four coronary artery disease. Nineteen were NYHA III or IV. Grade of AR was IV in 20, III in five and II in four. The STJ was dilated >50% of annulus diameter in each case (5.3-10.0 cm, mean 6.4 cm). All valves had three cusps. All patients underwent ascending aortic replacement. Seven had arch replacement and four coronary artery bypass. Seven had replacement of both right and non-coronary sinuses with re-implantation of the right coronary ostium. Twelve had replacement of the non-coronary sinus alone whilst nine had right coronary sinus replacement. One with dextrocardia had left coronary sinus replacement with ostial re-implantation. The graft size was within 2 mm of annulus size except for two patients (24 mm 12, 26 mm 11, and 28 mm six). Post operative echocardiographic studies were performed. None of the patients received anticoagulation. RESULTS: There were no hospital or late deaths and no thromboembolic or infective complications. Two patients had mild to moderate aortic regurgitation. These had a size 28 graft, which in retrospect was too large. Others had no significant regurgitation. CONCLUSIONS: The native aortic valve can be preserved in the majority of patients with AAA. Remodelling of the STJ and selective sinus replacement restores valve competence. Anticoagulation and prosthesis related complications are thereby avoided.     

Major venous sinus resection in the surgical treatment of recurrent aggressive dural based tumors

BACKGROUND: Despite gross total resection, aggressive dural based tumors invading major venous sinuses have high recurrence rates with poor long-term survivability. Options for aggressive surgical management of dural sinus invasion may be limited by the inherently high risk of morbidity and mortality. METHODS: Between July 1996 and July 2002, 5 cases of recurrent aggressive dural based tumors were operated on. Gross total resection had been previously performed in 4 cases, and near total resection in 1 case. Tumor pathology included 2 malignant meningiomas, 1 hemangiopericytoma, 1 atypical meningioma, and 1 benign meningioma with atypical features. All tumors recurred within 3 to 47 months and occluded a major venous sinus (four superior sagittal and one dominant right transverse sinus). Gross total resection of tumor and involved venous sinus was accomplished in each case. RESULTS: Three patients had no signs of clinical or radiographic recurrence at 10, 18, and 53 months of follow up. One patient who developed a fatal pulmonary embolism 10 months postoperatively had evidence of tumor progression on autopsy. One patient had tumor recurrence at 10 months, but is alive at 38 months and receiving adjunctive therapy. CONCLUSION: For aggressive dural based tumors that recur with invasion of a major venous sinus, radical resection of tumor and occluded sinus can be performed safely and may improve long-term survival.     

Transposition of the great arteries and isolated origin of the sinus node artery

We report the cases of 2 newborns who underwent at 7 days of age an arterial switch operation for transposition of the great arteries with a rare coronary anomaly: the left and right coronary arteries originated with a single ostium from sinus 1 and the sinus node artery had an isolated origin from sinus 2. The sinus node artery was reimplanted into the new aorta in both patients. Both babies were discharged in sinus rhythm. Preserving the vascularization of the sinus node may avoid the occurrence of postoperative atrial rhythm disturbances.     

Carotid Sinus Syndrome: Treatment by Carotid Sinus Denervation

Hypersensitive carotid sinus is a rare cause of spontaneous syncopal attacks. It must be differentiated from the other more common causes, such as intrinsic cardiac disease, vasovagal responses, postural hypotension and cerebrovascular insufficiency, although it may accompany these conditions. The definition of carotid sinus syncope is syncope elicited by stimulation of a hypersensitive carotid sinus.Nineteen patients with carotid sinus syncope were treated by carotid sinus denervation. Ages ranged from 48 to 83 with a mean of 65.5 years. Symptoms of marked dizziness or syncope were reproduced by gentle compression over the carotid bifurcation, while ECG monitoring revealed bradycardia or transient asystole. Seventeen patients had carotid arteriograms, eleven of which were normal. One patient had stenosis of the external carotid artery, while five had stenosis of the internal carotid. The right carotid sinus was involved in ten patients, the left in three and both sides in six. All patients underwent unilateral or bilateral carotid sinus denervation. Five patients with internal carotid stenosis had concomitant carotid endarterectomy. Complete relief of symptoms or marked improvement was noted in all but one patient. Postoperative follow-up ranged up to 15 years. Carotid sinus denervation is a simple, effective method of treating this disorder.     

Aneurysm of sinus of Valsalva

Aneurysm of sinus of Valsalva is a rare cardiac abnormality with congenital origin in most of the cases. If it is located in the right coronary sinus, it usually ruptures into a right heart chamber and frequently a ventricular septal defect (VSD) coexists with this condition. Early diagnosis and immediate surgical treatment can save the patient's life in most cases. All the 3 cases reported in this series had aneurysm of right sinus of Valsalva with associated VSD and mild degree of aortic regurgitation (AR). Two of the cases ruptured aneurysm into the right ventricle. Trans-esophageal echocardiography was used to confirm the diagnosis and all three showed good results with surgery.     

Invasion of the internal carotid artery by cavernous sinus meningiomas.

BACKGROUND: Meningiomas are the most common tumor involving the cavernous sinus. Although these tumors have been known to invade adjacent structures such as bone, soft tissue, and brain, invasion of the internal carotid artery (ICA) by meningiomas has only been recognized recently. The authors evaluate the extent of carotid wall involvement in nine patients with cavernous sinus meningiomas encasing the ICA who underwent en bloc resection of the cavernous sinus. METHODS: The en bloc tumor-ICA specimens were fixed in formalin, embedded in paraffin, and sectioned on a rotary microtome. Hematoxylin and eosin, EVG, and HVG stains were performed and evaluated by light microscopy. RESULTS: There were four males and five females with a mean age of 47 years. Eight patients had not undergone previous surgery, whereas one patient had been operated on before. In this latter case, however, the cavernous sinus was not entered during the first operation. In all patients, stenosis of the ICA was confirmed by preoperative angiography and/or magnetic resonance imaging (MRI). In seven cases, the tumors were excised en bloc along with the stenotic ICA segment. A petrous-to-supraclinoid ICA bypass was performed in these seven patients. In two cases, the tumor was excised with the stenotic artery, but no bypass was performed. The final pathological diagnosis was meningothelial meningioma. In all cases tumor cells were found in the adventitia of the cavernous carotid with stenosis of the arterial lumen. Compression and/or obliteration of the vasa vasorum within the adventia was noted in all specimens. In four cases, the tumor was found to have invaded the external elastic lamina. In two instances the external elastic lamina was disrupted and the tumor focally extended into the media. CONCLUSIONS: These findings suggest that in the case of cavernous sinus meningiomas with encasement and stenosis of the intracavernous ICA, invasion of the vessel wall has occurred. The effect of these findings on the management of cavernous sinus meningiomas and the involved ICA is discussed.     

Dural arteriovenous fistula of the transverse-sigmoid sinus causing trigeminal neuralgia

Summary The authors analysed an unusual case of dural arteriovenous fistula (DAVF) of the transverse-sigmoid sinus causing trigeminal neuralgia is presented. Although progression to almost continuous facial pain has been reported, symptoms may be indistinguishable from typical trigeminal neuralgia. The patient had a 6-year history of right-sided trigeminal neuralgia initially well controlled by medical management. He was referred for surgical management after 10 months of progressively worsening of symptoms. At the time of consultation, the patient complained of pulsatile tinnitus in the right ear. Computed tomography imaging and angiography demonstrated a DAVF involving the right transverse-sigmoid sinus junction with retrograde venous drainage. Surgical resection of the DAVF provided both angiographic cure and complete relief of all symptoms. The authors discuss the pathophysiology of trigeminal neuralgia in patients with a DAVF.     

Surgical repair of ruptured aortic sinus of Valsalva aneurysm

Surgical repair of ruptured aortic sinus of Valsalva aneurysm was performed on six patients. The NYHA functional class was I in one case, II in three and III in two cases. All aneurysms had ruptured into the right atrium. Three originated from the right, and three from the non-coronary aortic sinus of Valsalva. The preoperative shunt was 55-200% (mean 118%) of the peripheral cardiac output. At aneurysmal repair, closure of secundum-type atrial septal defect was performed in one case and insertion of a St Jude Medical aortic valve in another. There were no perioperative deaths. Five patients were asymptomatic in the follow-up period (5 months-17 years). One patient died of cardiomyopathy 11 years postoperatively. The long-term results after surgical repair of ruptured aortic sinus of Valsalva aneurysm thus were good, and early operation is recommended in order to avoid congestive heart failure.     

Cerebellar hemorrhage caused by dural arteriovenous fistula: a review of five cases

OBJECT: In this study the authors performed a retrospective analysis of five cases in which the patients (three women and two men) were treated for intracranial dural arteriovenous fistulas (AVFs) associated with cerebellar hemorrhage. On the basis of their findings, the authors evaluated the characteristics of this unusual symptom. METHODS: The dural AVFs were located in the right cavernous sinus in one patient, the left transverse-sigmoid sinus in three patients, and the right superior petrosal sinus (SPS) in one patient. All patients presented with severe headache and/or loss of consciousness. Computerized tomography scans revealed a small cerebellar hemorrhage near the fourth ventricle and hydrocephalus in four cases, and a massive hemispheric cerebellar hemorrhage in the remaining case. The four patients with small hemorrhages underwent ventriculostomy and endovascular treatment; all recovered. The patient suffering from a massive hemorrhage because of a dural AVF in the SPS was treated by suboccipital craniectomy, hematoma evacuation, and removal of the vascular anomaly. This patient remains in a persistent vegetative state. In four cases, results of angiography demonstrated retrograde leptomeningeal venous drainage through the SPS to the anastomotic lateral mesencephalic vein (ALMV) and/or to the vein of the lateral recess of the fourth ventricle (VLR4V). Retrograde leptomeningeal venous drainage to the ALMV and/or VLR4V was responsible for cerebellar hemorrhage in these cases. CONCLUSIONS: Thus, it is important to consider dural AVF in cases in which there is even a small hemorrhage near the fourth ventricle accompanied by intraventricular perforation and a decreased level of consciousness.     

Surgical Therapy of Arrhythmias in Single-Ventricle Patients Undergoing Fontan or Fontan Conversion

Abstract Background: Arrhythmia is detrimental to Fontan hemodynamics. Clinical outcomes among Fontan patients who underwent antiarrhythmic treatment were retrospectively reviewed. Methods: From January 1996 to January 2007, 182 patients underwent a Fontan procedure, including Fontan conversion. Thirty‐nine of the 182 patients showed various arrhythmias pre‐ or post‐Fontan operations, and were treated surgically including Fontan conversion (18 patients) or medically. The authors analyzed the outcomes of arrhythmia treatments retrospectively. Results: Thirty‐nine patients (21.4%) showed various arrhythmias, such as atrial flutter, atrial fibrillation, junctional rhythm, sinus node dysfunction, or brady tachyarrhythmia pre‐ or post‐Fontan procedure. Follow‐up duration was 13.1 ± 8.7 years (11 months to 325 months). Atrial flutter and fibrillation only developed in 17 patients who received atriopulmonary connection Fontan, and who were treated by Fontan conversion with concomitant procedures such as Cox‐maze procedure (two patients), right‐side maze and pacemaker implantation (five patients), right atrial isthmus ablation (four patients), right atrial isthmus cryoablation and pacemaker implantation (five patients), and only pacemaker implantation (one patient). The 21 patients who showed arrhythmia at the time of the Fontan procedure underwent the following procedures concomitantly: right atrial isthmus cryoablation with pacemaker implantation (one patient), right atrial isthmus cryoablation (one patient), or pacemaker implantation (nine patients). The remaining 10 patients, who showed junctional rhythm, sinus bradycardia, or intermittent ectopic beats, were managed medically. There were two late mortalities due to protein‐losing enteropathy. As a result, 33 patients (89.2%) maintained atrioventricular synchrony, 19 in sinus rhythm and 14 supported by a DDD‐type pacemaker. The remaining four patients (10.8%) showed persistent junctional rhythm with a stable hemodynamic status. Conclusions: The various arrhythmias in Fontan patients were well controlled by aggressive surgical management.     

主动脉窦瘤破裂12例外科治疗

本文报道12侧主动脉窦瘤破裂的外科治疗体会。表组合并室间隔缺损5例,主动脉关闭不全5例,细菌性心内膜炎2例。11例为右冠窦瘤破入右室或右房,1例为左和无冠窦瘤破入左室流出道。全组病例行直接或补片修补窦瘤,并补片修补室间隔缺损,对主动脉瓣关闭不全采取成形2例,2例主动脉瓣置换术。手术死亡1例。文中对其诊断、手术时机、心肌保护、手术方法进行了讨论。     

无顶冠状静脉窦综合征的外科治疗

目的分析无顶冠状静脉窦综合征（UCSS）的临床特点和外科手术治疗方法，为术中处理此类疾病提供借鉴。方法回顾性分析1998年5月至2008年1月在阜外心血管病医院手术治疗的44例UCSS患者的临床资料，术前诊断12例，术中诊断32例。根据Kirklin分型，其中Ⅰ型15例，Ⅱ型9例，Ⅲ型5例，Ⅳ型15例。44例均合并其他心脏畸形。合并左上腔静脉（LSVC）直接引流入左心房15例，其中手术采用心内隧道引流LSVC至右心房14例，直接结扎LSVC1例。手术同期矫治合并的其他心脏畸形。结果本组手术死亡3例，其中1例死于低心排血量综合征，2例死于肺部感染。1例因术后发生右侧膈肌麻痹行膈肌折叠术，术后70d出院。其余40例患者术后早期恢复顺利，术后呼吸机辅助时间11．7±12．1h；住院时间8．1±2．8d。随访32例，随访时间4个月～10年，其中9例左心房内隧道术处理LSVC患者，无死亡和并发症发生。结论先天性心脏病合并LSVC时，要警惕UCSS的存在。根据LSVC汇入左心房的位置选择不同的手术方法，可获得满意的手术效果。     

Valve replacement for Ebstein's anomaly of the tricuspid valve.

The role of valve replacement in the treatment of Ebstein's anomaly of the tricuspid valve remains controversial. Between 1965 and 1977, five patients with Ebstein's anomaly ranging in age from 11 to 57 years (mean 29) underwent tricuspid valve replacement in our institution. All of the patients were cyanotic but one, three were in Functional Class III, and two were in Class IV (New York Heart Association classification). The valve was placed above the coronary sinus. Plication of the atrialized portion of right ventricle was required in only one patient. Four patients had additional closure of an atrial septal defect. There were no deaths. On follow-up (range 1 to 13 years), four patients are in Functional Class I and one is in Class II. None is cyanotic. Four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. We conclude that valve replacement and, when necessary, plication of the atrialized right ventricle produce excellent clinical improvement.     

Long-term results after Fontan procedure and its modifications

The Fontan procedure is an effective method of treatment for patients with tricuspid atresia, univentricular heart, and other complex lesions. Modifications of the Fontan procedure have been developed to treat various anatomic malformations. From 1975 to 1984, 31 patients (17 male and 14 female) have undergone 35 Fontan operations. The youngest patient was 4 and the oldest 26 years of age (average 12.2 years). Sixteen patients had tricuspid atresia, and 15 univentricular heart. Twenty-three patients had undergone 37 palliative operations before the Fontan procedure. The original Fontan procedure was performed in 10 patients until 1977, and there were three early deaths (30%). After that time, the operative technique was modified and the early mortality dropped to 12% (3/25 patients). A valved prosthetic conduit was used in 11 patients (right atrium to pulmonary artery in five; right atrium to right ventricle in six), a valveless conduit in 14 patients (right atrium to pulmonary artery in nine; right atrium to right ventricle in five), and direct anastomosis (right atrium to pulmonary artery) in 10 patients. Postoperative cardiac catheterization was performed in 20 patients and revealed excellent results in 15. The remaining five had reduced oxygen saturation; three of them had had a Glenn anastomosis 8 and 10 years before the Fontan procedure, and one had an anomaly of the coronary sinus with a shunt to the left atrium. This patient required reoperation. Three other patients underwent reoperation because of calcified stenotic and/or outgrown conduits. Postoperative mean right atrial pressure varied from 10 to 20 mm Hg. The hemodynamic ventricular parameters were within the normal range. We conclude that in patients with tricuspid atresia, univentricular heart, and other complex cardiac lesions, inactivating the right ventricular pump function by means of the Fontan procedure does not adversely affect the systemic ventricle. Although the response to exercise was abnormal, the clinical condition of these patients was good to excellent.