Anterior chest wall deformities and congenital heart disease

Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.     

Surgical management of children and young adults with Marfan syndrome and pectus excavatum

Significant chest wall deformities occur in two thirds of children with Marfan syndrome (MS). The symptoms, physical findings, and surgical outcome of 11 patients with MS and severe pectus excavatum who required operative repair were reviewed. The diagnosis of MS was made before the pectus repair in six patients, at the time of evaluation of pectus in two patients, and after the repair in three patients. Symptoms included dyspnea upon exertion, tachypnea, and chest pain. Physical findings included aortic root enlargement or valvular disease, mitral valve disease, ligamentous disease, congestive heart failure, and ocular disease. All patients had severe pectus deformities with a narrow anteroposterior diameter in the midline, as well as a broad chest defect that extended bilaterally to the midclavicular line. The heart was shifted into the left side of the chest in all patients. A Ravitch-type pectus repair with a stainless steel substernal strut was used in eight patients, with one patient suffering a late recurrence; in three patients no strut was used, and all three had recurrence. There were no postoperative complications. In the postoperative follow-up of seven patients, symptomatic improvement of cardiopulmonary performance was noted. Four of the patients required subsequent open heart surgery, including replacement of the aortic valve in one patient, and composite grafts of the ascending aorta in three patients. Postoperative cardiac arrest was the only major open heart complication. All four patients recovered and did well after surgery, showing significant cardiac and pulmonary functional improvement.(ABSTRACT TRUNCATED AT 250 WORDS)     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.     

Simultaneous repair of pectus excavatum and congenital heart disease over the past 30 years

Objective: Pectus excavatum may be present in patient requiring operations for cardiac defects. The study was undertaken in order to assess our simultaneous repairs of pectus excavatum and congenital heart disease over past 30 years. Methods: Between 1970 and 2000, 12 patients underwent simultaneous repair of pectus excavatum and congenital heart disease. Six of 12 patients had ventricular septal defects as cardiac malformations (subgroup A). Operative technique, after the intracardiac procedure using cardiopulmonary bypass, consists of total subperichondrial resection of deformed costal cartilages, transection of deformed portion of the sternum in 2–3 points, and fixation of the sternum in elevated position using two Kirschner wires and a bridge external traction. Postoperative catheterization was performed in five of 12 patients (subgroup B). We evaluated the operative data, the improvement of pectus deformity and right ventricular performance retrospectively. The operative data in subgroup A were compared with those in recent random patients with ventricular septal defects only or with pectus excavatum only (control groups). Results: There was no operative death and non-serious complications were seen in nine patients (atelectasis in six, superficial wound infection in two, chylothorax in one). Pectus deformities improved with the drop of vertebral index postoperatively. The mean total operative time and postoperative drainage in subgroup A were 128.4% and 123.7%, respectively of those in the ventricular septal defect control group. The mean perioperative bleeding in subgroup A was more than the sum of those in control groups. Right ventricular end-diastolic (RVEDVI), end-systolic (RVESVI), stroke (RVSVI) volume indices and ejection fraction (RVEF) in subgroup B tended to increase after surgery. In particular, there were significant increases of RVEDVI (35%, P<0.05) and RVSVI (77%, P<0.01). Conclusion: Simultaneous cardiac and pectus repairs were performed successfully without serious complications. Moreover, simultaneous repair resulted in an improvement of right ventricular performance with significant increases of RVEDVI and RVSVI.     

Chest wall deformities

BACKGROUND: Pectus deformities and atypical costal anomalies are congenital thoracic wall defects that can cause a marked cosmetic defect with attendant psychological trauma and limited physical performance. PATIENTS AND METHODS: We reviewed 43 patients with chest wall deformities, 24 (55.8%) were pectus excavatum, 13 (30.2%) pectus carinatum and 6 (14%) atypical costal anomalies, in the last sixteen years. There were nine female and 34 (79.1%) male patients. The mean age of the patients was 14.4 years (range, 5 to 23). Scoliosis (13.5%), Poland's syndrome (5.4%), Marfan's syndrome (5.4%), neurofibromatosis (2.7%), atrial septal defect (2.7%) and mitral valve prolapse (13.5%) were associated with pectus deformities. The modified Ravitch's technique was used in pectus cases. Concomitant surgery was performed in two patients with pectus carinatum. RESULTS: The complications of pectus deformity repair were pneumothorax (24.3%), wound infection (8.1%), and local tissue necrosis (2.7%). There was no major recurrence, while minor recurrence rate was 10.8%. There was no mortality. CONCLUSION: Timely surgical procedures for the treatment of pectus deformities result in an excellent cosmetic outcome and improve cardiorespiratory function, providing both physical and psychological benefits.     

Surgical treatment of infective valve endocarditis in children with congenital heart disease

Abstract Objective: This study assesses surgical procedures, operative outcome, and early and intermediate‐term results of infective valve endocarditis in children with congenital heart disease. Methods: Seven consecutive children (five females, two males; mean age, 10.8 years) who underwent surgery for infective valve endocarditis between 2006 and 2010 were included in the study. The aortic and mitral valves were affected in two and tricuspid in five patients. Indications for operation included cardiac failure due to atrioventricular septal rupture, severe tricuspid valve insufficiency, and septic embolization in one, moderate valvular dysfunction with vegetations in three (two tricuspid, one mitral), and severe valvular dysfunction with vegetations in the other three patients (two tricuspid, one mitral). The pathological microorganism was identified in five patients. Tricuspid valve repair was performed with ventricular septal defect (VSD) closure in five patients. Two patients required mitral valve repair including one with additional aortic valve replacement. Results: There were no operative deaths. Actuarial freedom from recurrent infection at one and three years was 100%. Early echocardiographic follow‐up showed four patients to have mild atrioventricular valve regurgitation (three tricuspid and one mitral) and three had no valvular regurgitation. No leakage from the VSD closure or any valvular stenosis was detected postoperatively. Conclusions: Mitral and tricuspid valve repairs can be performed with low morbidity/mortality rates and satisfactory intermediate‐term results in children with infective valve endocarditis . (J Card Surg 2012;27:93‐98)     

Outcome of surgical closure of doubly committed subarterial ventricular septal defect

BACKGROUND: From 1986 to March 1997, 128 patients diagnosed to have doubly committed subarterial ventricular septal defects (VSD) were reviewed. Patients with aortic regurgitation (AR), and aortic valve (AV) deformity or a large left-to-right shunt across the VSD were offered operation. Forty-five patients (27 men, 18 women) agreed to surgical closure of their VSDs. METHODS: Thirty-eight patients had VSD closure alone, and 7 had an additional AV repair. Other associated defects corrected at operation were closure of atrial septal defects, closure of other ventricular septal defects, ligation of patent ductus arteriosus, and repair of ruptured sinus Valsalva aneurysm. RESULTS: There was no mortality nor major morbidity associated with operation. In the 26 patients with AR and AV deformity preoperatively, valve repair was performed in 6 patients. The condition of AR improved in 4, and remained unchanged in 22 patients. In the 10 patients with a deformity of the AV and no AR preoperatively, the condition remained unchanged in 5 patients, from whom 1 had valve operation, but progressed in 5 patients postoperatively at a mean follow-up of 6.4 years. In 9 patients with no deformity of the AV and no AR preoperatively, there was no postoperative AR and no progress of valve deformity. CONCLUSIONS: Excellent results were obtained with VSD closure and AV repair. Surgical closure of VSD, if performed before the onset of AV deformity, may prevent progressive AR. If AV repair is performed after the onset of AV deformity, progressive AR may not always be prevented.     

Fate of the aortic root after arterial switch operation.

OBJECTIVE: Concerns have been voiced about possible dilation and insufficiency of the neo-aortic valve after the arterial switch operation (ASO). AIMS: To determine growth of the neo-aortic valve and the aortic anastomosis after ASO and the prevalence of insufficiency or stenosis. PATIENTS AND METHODS: Since 1977, 144 consecutive patients (pts) underwent ASO for transposition of the great arteries (TGA). Median follow-up was 8.65 years (0.1--22.5 years). Simple TGA was present in 97 pts and 47 had TGA with ventricular septal defect (VSD). Detailed echocardiography included measurements of aortic diameter at four levels. The 608 measurements were compared with published normal values. RESULTS: The mean aortic valve z-score was 1.5, without significant change with age (P=0.75). Under 4 months, mean valve z-score was 0.63+/-2.20, between 5 and 12 months 2.56+/-2.30 (P<0.0001). Gradual growth occurs thereafter. The aortic sinus follows an identical growth pattern. The aorta at the anastomosis, is initially smaller than normal (z-score -0.64). After 4 months the z-score is 0.83, followed by continued growth of 0.1 z-score per year. At the last visit, the aortic valve z-score was above 2 in 51 patients, between -2 and 2 in 72 and less than -2 in six patients, none of whom had a flow velocity above 2 m/s. z-score of patients with VSD remained above those without VSD (P<0.0001).Aortic insufficiency was grade 2/4 in three patients, grade 3/4 in one and grade 4/4 in one. No patient developed aortic stenosis. CONCLUSION: After ASO the neo-aortic valve and sinus are larger than normal, representing the natural size difference in the prenatal situation and influence of associated cardiac malformations. In the first year of life, rapid dilatation of the new aorta is observed, followed by growth towards normalization of the valve and sinus size. Stenosis at the anastomosis was not observed. Aortic dilatation by itself is rarely associated with significant insufficiency.     

Emergent pectus excavatum repair after aortic root replacement in Marfan patient

Chest wall deformities, including pectus excavatum, can complicate cardiac operations by impeding sternal entry and cardiac exposure and by affecting cardiac hemodynamic performance. We describe a patient with Marfan syndrome who underwent elective replacement of an aortic root aneurysm. The patient required a simultaneous, unplanned, emergent repair of her severe pectus excavatum because of hemodynamic instability after sternal closure. The success of this procedure suggests that emergent single-stage repair of pectus excavatum should be considered when median sternotomy closure precipitates hemodynamic instability in such patients.     

Transcaval aortic valve implantation in a patient with Larsen syndrome: technical and anesthetic challenges

Transcatheter aortic valve implantation has emerged as a therapeutic option for patients with symptomatic severe aortic stenosis who are inoperable, or at very high risk of open-heart surgery. Recently, we encountered a patient with aortic stenosis and Larsen syndrome, who had short stature, obesity, kyphoscoliosis, multiple musculoskeletal deformities, and severe restrictive lung disease. An open-heart surgery in such a patient involves substantial peri-operative risk. A successful transcaval aortic valve implantation was done under general anesthesia.     

微创技术同期治疗漏斗胸合并先天性心脏病

目的 探讨应用微创技术同期治疗漏斗胸合并先天性心脏病(先心)的方法及可行性.方法 2006年7月至2011年6月应用双微创技术6例,其中男4例,女2例;年龄4～6岁5月,平均5岁4月;体重16 ～ 20 kg,平均(18.00±1.79) kg.CT Haller指数3.9 ～5.0,平均(4.35±0.43).其中4例行室间隔缺损微创伞封术(3例膜部和1例主动脉瓣下室间隔缺损,缺损直径4 ～5 mm);2例行中央型继发孔房间隔缺损微创伞封术,直径12～16mm.先心微创术后行Nuss手术,术后常规放置心包纵隔引流管.结果 手术顺利,术后5～11h拔除气管插管,平均(8.17±2.04)h.48h拔除心包纵隔引流管.无手术死亡、大出血及胸腔脏器损伤等危险并发症.术后检查先心封堵效果良好,肺复张良好.术后出现1例切口延期愈合,经治疗后,均顺利出院.3例行钢板取出术,效果满意.结论 微创技术同期治疗合并先心的漏斗胸安全、满意,避免了二次手术所带来的困难和风险.     

The incidence of Marfan syndrome and cardiac anomalies in patients presenting with pectus deformities

PurposeThe incidence of Marfan syndrome in the general population is 0.3%. Two-thirds of patients with Marfan syndrome have concurrent pectus deformity. However, incidence of Marfan syndrome and cardiac abnormalities in patients presenting with an isolated pectus deformity remains unknown. We sought to establish the degree of association between pectus deformities and these abnormalities, and whether referral of these patients for cardiac and genetic workup is warranted.MethodsOur pediatric surgery group refers patients with pectus deformities for genetic and cardiac evaluation. We examined 415 records from 2009 to 2016, and identified 241 patients with a chief complaint of a pectus deformity. Patient characteristics, echocardiogram results, Haller indices, and genetic results were analyzed.ResultsThe frequency of Marfan syndrome in our study was 5.3%. The incidence of Marfan was highest among patients with combined type pectus deformity (20%). Cardiac anomalies showed an overall incidence of 35%. Of those diagnosed with Marfan, 84% had cardiac abnormalities.ConclusionMore than 5% of patients presenting with a chief complaint of pectus deformity will have a diagnosis of Marfan syndrome, compared to 0.3% in the general population. Approximately a third of this population will have cardiac abnormalities. Referral of patients with pectus deformities for evaluation for Marfan syndrome and cardiac abnormalities is appropriate.Level of EvidenceLevel IV.     

A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair

BACKGROUND: We reviewed our operative experience and long-term results with repair of pectus excavatum and carinatum deformities through a vertical midline approach, including those cases with simultaneous intracardiac repair. METHODS: From 1972 through 1998, 120 children underwent pectus deformity repair. Operative technique used a vertical midline incision with subperichondrial resection of deformed cartilages and an anterior sternal osteotomy. Thirty-five patients had a temporary metal bar for retrosternal support for 6 months; 85 underwent repair without a bar. Patients and parents were asked to assess the outcome after pectus repair as poor, fair, good, or excellent. RESULTS: There were 94 male and 26 female patients (mean age, 8.4 years; range, 3 to 21 years). There were 111 cases of pectus excavatum and 9 of pectus carinatum. Fourteen children (11.5%) had an associated congenital heart defect; 9 patients had simultaneous pectus and intracardiac repair. One patient was referred for emergent open heart repair and pectus repair after attempted "Nuss" repair resulted in a perforated right atrium, perforated right ventricle, and partially disrupted tricuspid valve apparatus. There were no deaths and only one significant complication, which required a return to the operating room for bleeding. Morbidity was not higher in patients with simultaneous intracardiac repair. Long-term follow-up was established in 83% of patients. Results were classified as excellent in 64 patients (64%), good in 25 (25%), fair in 8 (8%), and poor in 3 (3%). Thirty (86%) of 35 patients with a sternal bar had excellent results versus 34 (52%) of 65 without a bar (p = 0.004); 97% of patients who underwent repair with a sternal bar classified the result as excellent or good. CONCLUSIONS: Long-term results of pectus excavatum and carinatum repair through a vertical midline approach are excellent. Outcome with a temporary sternal bar is superior to outcome without a bar. Concomitant repair of congenital heart defects and pectus deformity may be performed successfully without additional morbidity.     

Ventricular septal defect and aortic insufficiency: surgical considerations and results of operation

Ventricular septal defect (VSD) is uncommonly associated with valvular aortic insufficiency (AI). From 1957 through 1971, 51 patients with this combination underwent operation at the Mayo Clinic; these patients made up 2.2% of all patients with VSD repaired during that period. The most frequent aortic valve abnormality was a prolapsing right cusp. Surgical management of AI consisted of: (1) no procedure on the aortic valve in 25 patients, (2) plastic repair of the aortic valve in 13 patients, and (3) partial or total replacement of the aortic valve in 13 patients; in the group with severe AI prior to operation, those treated by replacement did better than those treated by repair. Early closure is indicated for VSD associated with mild AI. With severe AI, VSD closure and valve plication are preferred, although valve replacement may be necessary either at the primary operation or at some future date. In adults, replacement with a prosthesis is indicated for severe AI.     

The long-term outcome of a surgical repair of sinus of valsalva aneurysm

BACKGROUND: In order to clarify the long-term outcome after surgical repair of a sinus of Valsalva aneurysm, we retrospectively assessed the operative results for patients treated in our institute. METHODS: The subjects were 27 patients who had undergone an operation between 1958 and 1996. For associated aortic regurgitation (AR) aortic valve repair was performed in 13 patients, 12 of whom had a ventricular septal defect (VSD); and an aortic valve replacement was performed in 3 patients, 1 of whom had a VSD. RESULTS: Five of the 13 patients who had aortic valve repair needed aortic valve replacement because AR developed after a period of between 7 and 13 years; those cases were complicated by VSD. Another 2 patients with mild AR also complicated by VSD are currently under observation. CONCLUSIONS: Although the postoperative outcome of the aortic valve repairs was good, cases that were complicated by VSD plus associated AR tended to develop AR later after surgery. Therefore, careful observation of the postoperative course is necessary.     

Rupture of the inferior vena cava during open-heart surgery. Aetiology and surgical management

Bleeding during open-heart surgery is a not unusual complication. Rupture of the inferior vena cava during dissection of the heart from adhesions from a previous operation for mitral disease or congenital palliative surgery is a serious and sometimes fatal complication. Its treatment is a challenge even to the most experienced heart surgeon. In two of our three patients, vena cava rupture occurred during open-heart surgery for correction of mitral and aortic valvular disease. In one patient, congenital infundibular stricture of the right ventricle, associated with tricuspid valve insufficiency, was present. In this report, some relevant data were presented from each case and the aetiology of the rupture is discussed.     

Ventricular septal defect and aortic insufficiency. Surgical treatment.

Twenty-five patients with ventricular septal defect (VSD) associated with aortic insufficiency (AI) have been since 1964. Of these patients, one died suddenly without operation; in 2 patients, AI developed in the late postoperative period following VSD closure; and in 3 others, AI developed shortly after VSD closure. The remaining 19 patients are discussed in detail. The VSD was subpulmonic in 13 (68 per cent) and subcristal in 6 (32 per cent). Primary suture of the VSD was undertaken in 13 patients and patch closure in 6. Seven patients had aortic valvuloplasty and 2 had aortic valve replacement. There were no surgical deaths, and the long-term follow-up shows that VSD closure alone has been sufficient to arrest progression of AI in patients with mild insufficiency, particularly in those with subpulmonic VSD. Valvuloplasty, when necessary, was more effective when done at an early age.     

50例先天性主动脉窦瘤的外科治疗

总结先天性主动脉窦瘤外科治疗经验。方法：先天性主动脉窦瘤５０例,合并室间隔缺损３１例,主动脉瓣脱垂１４例。行窦瘤切除修补术１７例,窦瘤切除加定夺缺修补１９例,窦瘤切除加主动脉瓣成形１２例,主动脉瓣替换２例。结果：全组无手术死亡,无残余分流。结论：心脏超声对ＣＡＳＶ诊断准确率高。     

Aneurysm of the ascending aorta in its coarctation

In long-term period after resection of aorta coarctation, 9-14% patients developed aortic aneurysms, one third of them localized in the ascending aorta. From 146 patients operated on for aneurysm of the ascending aorta, 3 had aortic coarctation. In 2 patients aneurysms of the ascending aorta formed late after resection of the coarctation, the third patient was hospitalized with clinical picture of cardiac insufficiency at terminal stage due to coarctation of the aorta and a giant aneurysm of the ascending aorta with significant aortic insufficiency. The patients underwent successful surgeries: 1) ascending aorta grafting; 2) aortic valve and ascending aorta grafting by Bentallo de Bono method; 3) aortic valve and ascending aorta grafting by Bentallo de Bono method with bypass of descending aorta from the conduit. Cystic medianecrosis and two-volume aortic valve were revealed in all the patients. It is concluded that patients after surgery for coarctation of the aorta require long-term follow-up to defect cardiovascular complications early.     

Infective endocarditis with congenital heart disease

From 1988 to 2005, seven patients were operated at our hospital because of infectious endocarditis (IE) with congenital heart disease (CHD). Underlying CHD included ventricular septal defect (VSD) in 4 (2 previous operations with residual region), atrial septal defect (ASD) in 2 and bicuspid aortic stenosis (AS) in 1. No cases had preventive antibiotic prophylaxis for dental procedures. We could confirm bacteria origin from blood culture in all cases, but two patients had operations without gaining control of the infection. VSD or ASD closure and valve surgery were performed in four patients. One patient had a VSD closure, two patients had valve surgery. There were no operative or hospital deaths and there were no recurrences of IE during the study period. We successfully treated IE with CHD by enough debridement of the infective focus of IE, and valve surgery. It is important for patients with CHD to have preventive antibiotic prophylaxis for dental procedures.