Associated factors and trends in outcomes of interrupted aortic arch

Background

Interrupted aortic arch (IAA) continues to be associated with important mortality, both before and immediately after repair, with ongoing morbidity during follow-up. We sought to determine trends in presentation, management, outcomes and associated factors.

Methods

We reviewed all consecutive patients (n = 119) presenting from 1975 to 1999, and data were collected regarding demographics, anatomy, management and outcomes.

Results

Significant trends over time for patients born in three consecutive periods (1975 to 1984, 1985 to 1993, and 1994 to 1999) demonstrated a smaller proportion of patients with presentation with circulatory collapse (65%, 51%, and 25%, respectively), greater use of prostaglandins (72%, 90%, 100%), fewer deaths without IAA repair (49%, 15%, 13%) and greater use of one-stage repair (68%, 75%, 100%). Independent risk factors for death without IAA repair (p < 0.001) included absence of ventricular septal defect, and the presence of noncardiac anomaly, complex cardiac anomaly, episode of acidosis and earlier birth cohort. Overall survival after repair was 50% at age 1 month, 35% at 1 year, and 34% at 5 years. Early and constant-hazard phases were noted, with incremental risk factors for early phase mortality being cyanosis at presentation, presence of truncus arteriosus or aortic stenosis, an episode of circulatory collapse before repair, earlier date of repair, and lower weight at repair. Greatest survival occurred in those patients with uncomplicated IAA who had repair since 1993 (5 year survival, 83%). Freedom from reintervention for arch obstruction was 60% at 5 years.

Conclusions

While improving, outcomes of IAA remain of concern, especially in patients with associated lesions.     

Determinants of early and late outcome for reoperations of the proximal aorta

Background

The purpose of this study was to investigate the cause of ascending aorta and aortic arch reoperations and to identify determinants of early and late outcome.

Methods

Between January 1991 and March 2003 we repaired aneurysms of the proximal aorta in 597 patients. Of these patients, 104 had reoperations for replacement of the ascending aorta, aortic root, or transverse aortic arch. Previous surgery was defined as any previous cardiac or proximal aortic repair. Median age was 60 years, and 29 of the patients (28%) were female. Indications for reoperation and replacement of the proximal aorta included acute type A dissection in 6 patients (5.8%), aneurysm with chronic dissection in 60 (57.7%), progression of aneurysm in 23 (22.1%), infection in 12 (1.5%), inflammatory disease in 2 (1.9%), and atheromatous disease in 1 (1.0%). Reoperations included aortic root replacement in 20 patients (19.2%), total arch replacement with elephant trunk in 28 (26.7%), ascending and proximal arch in 39 (37.5%), and ascending aorta in 27 (26.0%). The median interval between operations was 69 months. Retrograde cerebral perfusion was used in 80 (77%) cases.

Results

Chronic dissection was the most common indicator for reoperation in our population, followed by progression of aneurysm and infection. Thirty-day and in-hospital mortality was 13.5% (14 of 104) and 15.4% (16 of 104), respectively. Chronic obstructive pulmonary disease, renal dysfunction, and increased pump time were risk factors for mortality. Median follow-up was 5.02 years. Eight patients died during that period. Estimated survival at 1, 5, and 10 years was 83%, 80%, and 62%, respectively. Freedom from second proximal reoperations was 97.1% (10 of 104). Freedom from subsequent distal thoracic aortic repair was 84.6% (8 of 104).

Conclusions

Reoperations of the ascending aorta and aortic arch can be performed safely with good long-term results. Patients with previous proximal aortic dissection repair need long-term surveillance. Renal dysfunction and chronic obstructive pulmonary disease must be carefully considered before reoperations of the proximal aorta.     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.     

One stage repair of aortic arch anomalies and intracardiac defects

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical complications in relation to functional outcomes after ileoanal anastomosis in pediatric patients with ulcerative colitis

Background

Significant surgical complications are common in patients with ulcerative colitis who undergo proctocolectomy. We assessed the effects of these complications on the functional outcomes of such patients.

Materials and Methods

The medical records of 47 consecutive patients who underwent ileoanal anastomosis (IAA; J-pouch IAA, n = 37; straight IAA [SIAA], n = 10) for ulcerative colitis between 1985 and 2004 at a median age of 13.7 years (range, 5.5-19.3 years) were reviewed. Anastomotic leakage and stenosis, fistula, intestinal obstruction (IO), wound complications, pouchitis/enterocolitis (no/once/recurrent), permanent ileostomy, and ultimate diagnosis of Crohn's disease were recorded and correlated with the functional outcome measures of stooling frequency (6 months, 2 years, present), present incidence of soiling, and continence score (range, 0-12).

Results

The median postoperative follow-up period was 10 years (range, 1-21 years). Twenty-six (55%) of the 47 patients had surgical complications (J-pouch IAA, 21/37 [57%]; SIAA, 5/10 [50%]; P = nonsignificant). These complications specifically included stenosis of IAA in 4 (9%) patients (SIAA 2), pelvic abscess/sepsis in 4 (9%) patients, late fistula in 4 (9%) patients (SIAA 1), early IO in 8 (23%) patients (SIAA 1), late IO in 14 (27%) patients (SIAA 3), J-pouch prolapse in 1 patient, and wound complications in 7 (15%) patients (SIAA 1). Twenty-three (49%) patients had pouchitis, which recurred in 13 (28%). Ileoanal anastomosis stenosis, IO, J-pouch prolapse, wound complications, and pouchitis did not worsen functional outcomes. In 4 patients with pelvic sepsis, early stooling frequency and the continence score were lower than those in the rest of the patients (P < .05). Crohn's disease was ultimately diagnosed in 3 (6%) patients (SIAA 1), 2 of whom (SIAA 1) had permanent ileostomy formation.

Conclusions

Significant surgical complications are common after IAA. Complications do not generally worsen functional outcomes, except in those patients with septic complications or the ultimate diagnosis of Crohn's disease.     

Critical aortic stenosis in early infancy: surgical treatment for residual lesions after balloon dilation

Background

The optimal management for critical aortic stenosis in early infancy continues to challenge cardiologists and cardiac surgeons. We present a review of our experience with the surgical treatment of residual aortic valve disease after percutaneous balloon dilation for critical aortic stenosis in early infancy.

Methods

Since 1989, 11 of the 38 patients who survived aortic balloon dilation (28.9%) have undergone surgical treatment for residual aortic valve dysfunction. Median time from aortic balloon dilation to surgical intervention was 7 months (range 1 to 56 months). Residual aortic stenosis was the predominant problem in 8 patients and aortic regurgitation was predominant in 2 patients.

Results

Aortic valvuloplasty was possible in 5 children; pulmonary autograft replacement of the aortic valve was performed in 6 children. Two children underwent a Ross-Konno procedure because of annulus hypoplasia and severe left ventricular outflow tract obstruction. Two early deaths occurred after a Ross-Konno procedure, both with findings of severe left ventricular fibroelastosis at the pathologic examination. Median follow-up time was 5 years (range 1 month to 11.9 years). No late deaths occurred. One patient with moderate-severe aortic valve regurgitation after aortic valvuloplasty underwent a successful Ross operation. All 9 patients are asymptomatic and are in good clinical condition.

Conclusions

We are convinced that the best aortic valve in the pediatric age group is the native one, provided it can function acceptably. However, in cases where conservative surgical treatment fails to yield a functional aortic valve, replacement of the valve is indicated, and the best aortic valve substitute in infants is the pulmonary autograft because of its potential for growth.     

Anatomic reconstruction for recurrent aortic obstruction in infants and children

Background

Patients undergoing operative repair of aortic obstruction are at a lifelong risk of recurrent obstruction, and there is controversy regarding the optimal surgical technique. We have used an alternative strategy for recurrent aortic obstruction, typically involving anatomic reconstruction by means of a median sternotomy, and describe our techniques and results.

Methods

Twenty-one patients presented with recurrent aortic arch obstruction. Mean age and weight were 7.8 ± 5.4 years (range, 0.21 to 15.2 years) and 30.6 ± 21.8 kg (range, 3.6 to 90 kg), respectively. Recurrence involved the aortic arch to some degree in each case, as the mean preoperative transverse aortic arch z score was −2.9 ± 1.6 (range, −7.0 to 0.1). Thoracotomy was possible in 2 patients, using re-resection with end-to-end anastomosis (n = 1) and patch aortoplasty (n = 1). The remaining 19 patients required median sternotomy, cardiopulmonary bypass, and deep hypothermic circulatory arrest for complete relief of obstruction by aortic arch advancement (n = 10), patch aortoplasty (n = 8), or interposition grafting (n = 1).

Results

There was 1 hospital death. Invasive blood pressure monitoring revealed no residual arm-to-leg gradient in 19 patients and a 20-mm Hg gradient in 2 patients. There have been no late deaths. No patients have undergone subsequent aortic intervention, and all are asymptomatic up to 85 months postoperatively. Two patients are currently followed with a 10-mm Hg arm-to-leg blood pressure gradient.

Conclusions

Anatomic reconstruction for recurrent aortic obstruction can be safely accomplished in the majority of patients. We favor median sternotomy because of the ability of establishing cardiopulmonary bypass, the facility of anatomic reconstruction techniques, and the ability to repair concomitant cardiovascular lesions.     

Initial results after combined repair of aortic arch aneurysms by sequential transposition of the supra-aortic branches and consecutive endovascular stent-graft placement

Background

To evaluate safety and efficacy of a combined repair of aortic arch aneurysms by sequential transposition of the supra-aortic branches and endovascular stent-graft placement.

Methods

Between October 2002 and September 2003, 5 patients (mean age, 79.5 years) presented with aortic arch aneurysms involving the origin of the left carotid artery. Treatment was made by sequential transposition of the left carotid artery into the brachiocephalic trunk and transposition of the left subclavian artery into the already transposed left common carotid artery with consecutive endovascular stent-graft placement into the aortic arch.

Results

All patients survived both procedures. At completion angiography, a small type 1a endoleak was observed in 1 patient. After 1 week, the patient was readmitted for completion three-dimensional computed tomographic scan. The leak had already occluded spontaneously. Mean follow-up was 10 months (range, 4 to 16 months). At follow-up, all patients had normal computed tomographic scans with regular perfusion of the supra-aortic branches without any signs of endoleaks.

Conclusions

Combined repair of aortic arch aneurysms by sequential transposition of the supra-aortic branches with consecutive endovascular stent-graft placement is feasible. Extended application of this technique will enable safe and effective treatment of a highly selected subgroup of patients with aortic aneurysms by avoiding conventional arch aneurysm repair in deep hypothermia and circulatory arrest.     

Long-term follow-up of patients with high-risk congenital diaphragmatic hernia

Background/Purpose

Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established.

Methods

Thirty-three children with ages 4.1 ± 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy.

Results

No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung.

Conclusion

These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.     

Outcome after aortic valve replacement in octogenarians

Background

The advancing age of the population in the western world and improvements in surgical techniques and postoperative care have resulted in an increasing number of very elderly patients undergoing cardiac operations. Therefore, the aim of this study is to evaluate the surgical outcome in 115 octogenarians after aortic valve replacement.

Methods

We retrospectively identified 115 patients (47 men, 68 women) aged 82.3 ± 2.1 years (mean, 80 to 92 years) who underwent aortic valve replacement alone (71 patients, 62.1%) or in combination with coronary artery bypass grafting (44 patients, 37.9%), between January 1992 and April 2003. These patients had significant severe aortic stenosis with a mean valve area of 0.62 ± 0.15 cm² and a mean gradient of 88.62 ± 24.06 mm Hg.

Results

The in-hospital mortality rate was 8.5%. The late follow-up was 100% complete. Actuarial survival at 1 and 5 years was 86.4% and 69.4%, respectively. Predictors of late mortality were ejection fraction (p < 0.01), preoperative heart failure (p < 0.03), and the type of prosthesis (p < 0.03).

Conclusions

The outcome after aortic valve replacement in octogenarians is excellent; the operative risk is acceptable and the late survival rate is good. Therefore, cardiac surgery should not be withheld on the basis of age alone.     

Neurocognitive functions after aortic arch repair with right brachial artery perfusion

Background

Satisfactory neurologic outcome following aortic arch repair through right brachial artery perfusion is well established. However, how neurocognitive functions are affected following selective cerebral perfusion, still needs to be elucidated.

Methods

In a period between April 2002 and March 2003, 22 patients (19 male, 3 female, with a mean age of 46.8 ± 12; range: 26 to 70 years old), underwent aortic arch repair using right brachial artery low flow (8 to 10 mL · kg⁻¹ · min⁻¹) selective antegrade cerebral perfusion under moderate hypothermia (26°C). There were 6 Stanford type-A dissections and 16 ascending aortic aneurysms. All patients were evaluated preoperatively and postoperatively (at seventh day and second month) for neurocognitive functions.

Results

There was no operative mortality. The average cardiopulmonary bypass time was 115.0 ± 24.2 minutes and the average antegrade cerebral perfusion time was 29.8 ± 7.1 minutes (19 to 38 minutes). No major neurologic deficit was observed in the postoperative period. In terms of neurocognitive test results, between the preoperative and postoperative assessments for both hemispheric cognitive functions no deterioration was detected.

Conclusions

The low-flow selective antegrade cerebral perfusion technique through the right brachial artery may safely be used for the great majority of patients undergoing aortic arch repair without causing deteriorations in neurocognitive functions.     

Closure of ventricular septal defect of interrupted aortic arch with left ventricular outflow obstruction]

Leftward shift of the infundibular septum (IS) in interrupted aortic arch (IAA) with ventricular septal defect (VSD) often develops significant left ventricular outflow obstruction (LVOTO). Seven-day-old boy with 2.6 kg body weight underwent the two-stage operation for this anomaly. The aortic arch was interrupted between the left common carotid and the left subclavian artery. At the first stage, a 5 mm GORE-TEX graft was used to connect the interrupted arch, and pulmonary artery banding was performed. In closure of VSD at the second stage, IS was penetrated by stitches for the VSD patch to left ventricular outflow tract. IS with leftward shift could be pulled toward right ventricular side with patch fixation and LVOTO was prevented by this method.     

Primary repair of aortic arch obstruction with ventricular septal defect in preterm and low birth weight infants

Objective: Previous reports have suggested that prematurity and low birth weight are risk factors for definitive surgical intervention in congenital cardiac malformations. The following data review our experience with primary repair of the complex malformation of aortic arch obstruction with ventricular septal defect (VSD) in this patient population. Methods: Since 1988, 21 consecutive preterm (≤36 weeks) and/or low birth weight (<3000 g) infants with interrupted aortic arch (IAA; n=10), or aortic coarctation (n=11) with VSD, underwent primary arch repair and VSD closure. The mean weight at operation was 2310 g (range, 1200–2900 g), including 12 patients at ≤2500 g. The gestational age ranged from 30 to 41 weeks (mean, 36.4 weeks). Five patients with interrupted arch and two patients with coarctation also had severe subaortic stenosis, which was relieved by transatrial incision of the infundibular septum. Results: The overall hospital mortality was 14% (3/21). Death was related to low cardiac output in association with severe subaortic stenosis (n=2) and sepsis (n=1). Late mortality occurred in three patients, two of which were non-cardiac. The mean follow-up was 33 months. Two patients had significant recurrent arch obstruction, which was successfully relieved by balloon angioplasty and surgical correction in one each. The survival at 30 days, and at 1 and 3 years was 86, 76 and 70%, respectively. Conclusions: Complete primary repair of aortic arch obstruction with VSD can be achieved with good results, even in the preterm and low birth weight infant. Therefore, early surgical repair of this congenital malformation is recommended.     

Outcome analysis of major cardiac operations in low weight neonates

Background

From June 1995 to January 2003, 49 consecutive neonates of less than 2,500 g underwent early surgery for congenital heart disease. A retrospective analysis was performed to evaluate the early to medium term outcome.

Methods

Major cardiac surgery for congenital heart defects included a complete correction in 31 patients (group I) and a palliative procedure in 18 patients (group II). Mean age at operation was 15.2 days (1 day-90 days) and mean weight was 2,190 g (1,300 g-2,500 g). Twenty-four children (49%) were born prematurely. All neonates were critically ill and 47% were already ventilated preoperatively. Heart defects included mainly ventricular septal defect (10), tetralogy of Fallot complexes (8), aortic coarctation (8), transposition complexes (7), single ventricle anomalies (4), pulmonary atresia with intact septum (4), interrupted aortic arch (3), totally anomalous pulmonary venous return (3), and common atrioventricular septal defect (2).

Results

Overall surgical mortality was 18%: 4 neonates died after definitive repair and 5 after palliation; representing, respectively, 13% and 28% of each group. Postoperative morbidity occurred in half of the patients (53%). Age, weight, prematurity, type of first surgical procedure, and use of cardiopulmonary bypass did not influence the early outcome. After a mean follow-up of 2.82 years (2 months to 6 years), survival was 87% in the correction group and 54% in the palliation group. All children were in NYHA class I-II. Freedom from reintervention at 18 months was 68% after correction versus 8% after palliation.

Conclusions

Cardiac surgery for congenital malformations in critically ill, low weight neonates can be achieved with acceptable mortality, at the cost of an increased morbidity. Early outcome seems independent of age, weight, prematurity, use of extracorporeal perfusion, and type of first intervention. Moreover, primary correction appears to result in an early survival benefit, remaining constant over time.     

Interruption of the aortic arch. Surgical considerations.

During a 10 year period, January, 1965, through January, 1975, 5 patients with interruption of the aortic arch (IAA) underwent operation at the Texas Heart Institute. The mortality rate was 60 per cent; 2 patients survived the operation. One 11-day-old infant with IAA, type A, a ventricular septal defect (VSD), and a patent ductus arteriosus (PDA) underwent successful two-stage treatment. A left subclavian-ductus anastomosis, closure of the PDA, and banding of the pulmonary artery were done initially. The VSD was closed later. The second survivor, a 3-year-old girl, had IAA, type B, with a PDA and VSD. Total correction was done with the aid of cardiopulmonary bypass and hypothermia. Considerations include palliative and staged procedures versus total correction with either conventional cardiopulmonary bypass or deep hypothermia and circulatory arrest. Survival rate is improved if associated lesions are totally repaired or palliated at the time of reconstruction of IAA.     

Pilomatrixoma--accuracy of clinical diagnosis

Background/Purpose

Pilomatrixoma is a common tumor of skin appendages in children. The aim of the study was to assess the accuracy of clinical diagnosis and factors contributing to misdiagnosis.

Methods

A retrospective case note review of patients who had pilomatrixoma excised during a 5-year period in a tertiary referral children's hospital in the UK.

Results

From 75 patients, 78 pilomatrixomata were excised. The diagnosis was achieved preoperatively in 46% of patients. Other diagnoses included sebaceous and dermoid cysts, foreign body reaction, calcification in lymph gland, and fat necrosis.

Conclusion

Factors contributing to misdiagnosis include cystic lesions with varying consistency, punctum-like appearance, atypical location, and absence of clinically recognizable calcification. Despite close excision, the recurrence rate is low.     

Impact of slit and nonslit mesh technique on testicular perfusion and volume in the early and late postoperative period of the totally extraperitoneal preperitoneal technique in patients with inguinal hernia

Aims

Using slit and nonslit mesh in laparoscopic totally extraperitoneal preperitoneal (TEPP) inguinal hernia repair are well-known approaches. The aim of this prospective, randomized, clinical study was to assess testicular perfusion after these procedures.

Methods

In the study period, 40 male patients with unilateral inguinal hernia were assigned into 2 equal groups as follows: slit (S) and nonslit (NS). TEPP hernia repair was performed in all patients. In the 2 groups, testicular arterial blood flow and testis volumes were measured by Doppler ultrasonography preoperatively, on the 5th postoperative day, and 6 months postoperatively, respectively.

Results

No statistically significant difference was found between the preoperative, 5th day postoperatively, and 6-month arterial resistance index (ARI) results when comparing the S and NS groups in ultrasonographic testicular blood flow studies. There was no statistically significant difference of testicular volume between the preoperative period, the 5th postoperative day, and 6 months postoperatively in the 2 groups.

Conclusions

According to the results, no statistically significant difference was found in terms of testicular perfusion and volume between those 2 methods of TEPP repair for inguinal hernia.     

Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group

Background/Purpose

Patients with congenital diaphragmatic hernia (CDH) are known to have associated cardiac anomalies. Data from the Congenital Diaphragmatic Hernia Study Group has allowed better definition of the types of heart defects (HDs) and survival for these patients.

Methods

Since 1995, 2636 patients were enrolled in the Congenital Diaphragmatic Hernia Study Group from 82 centers. Patients with hemodynamically significant HD, excluding patent ductus arteriosus (PDA); patent foramen ovale (PFO); and atrial septal defect (ASD), were selected. Cardiac anatomy and survival data for all patients were reviewed.

Results

Two hundred eighty (10.6%) patients had significant HDs: ventricular septal defect (VSD); (42.2%), aortic arch obstruction (15%), univentricular anatomy (13.9%), tetralogy of Fallot variants (11.1%), total anomalous pulmonary venous return (3.9%), double outlet right ventricle (RV) (3.2%), pulmonary stenosis (2.5%), transposition of the great arteries (2.5%), and various other defects in 5.7%. Survival for the entire group was 67.1%; survival for patients without HD was 70.2% and for patients with HD was 41.1% (P < .001). Patients with biventricular cardiac anatomy had a 47% survival, whereas those with univentricular anatomy had a 5% survival (P < .001).

Conclusion

Significant HD is associated with 10.6% of CDH. Survival for patients with HD is significantly lower than for patients with normal cardiac anatomy. Patients with CDH and univentricular cardiac anatomy have a poor prognosis.     

Femoral cannulation is safe for type A dissection repair

Background

Recently, surgeons have embraced axillary artery cannulation for type A aortic dissection repair out of concern for malperfusion phenomena with traditional femoral artery cannulation. My colleagues and I sought to determine whether these concerns are justified.

Methods

Records of 86 consecutive patients (51 men and 35 women; age, 30 to 86 years; mean, 62 years) undergoing surgical repair for acute type A dissection were reviewed. Cannulation site, specific operative repair, and complications related to cannulation were noted.

Results

Seventy-nine cannulations were performed in the femoral artery (47 left, 23 right, and 9 unspecified), 3 in the axillary artery (1 left and 2 right), and 4 in the ascending aorta or arch. Deep hypothermic arrest was used in 64 operations. Seven involved re-sternotomy. Seventy patients had supracoronary grafts (2 with valve replacement and 10 with valve resuspension), and 16 underwent aortic root replacement. Fourteen patients were in shock from cardiac tamponade. Eighty patients survived the operation, and 71 were hospital survivors. Malperfusion on initiation of cardiopulmonary bypass was noted in 3 patients. In 1, the original cannulation site was the ascending aorta, and the cannula was moved to the femoral artery for correction. In 2, the original cannulation site was the femoral artery, and the cannula was moved to the ascending aorta. Malperfusion on clamping of the aorta or on resumption of aortic flow was noted in no patient. Postoperative ischemia of any vascular bed was noted locally only in 3 (cannulated) lower extremities.

Conclusions

Straight femoral cannulation for all phases of type A dissection repair is appropriate and yields excellent clinical results. The anticipated malperfusion events are actually rare (2 of 79 with femoral artery cannulation, or 2.5%).