Repair Of Congenital Cardiac Malformations Research Articles

Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis.

Sickle-cell patients undergo cardiopulmonary bypass (CPB) surgery in our institution without perioperative exchange transfusion. We sought to determine whether this protocol increased mortality or important sickle-cell-related complications. We adopted a 1:1 matched-pair case-control methodology to evaluate the safety of our protocol. Sickle-cell patients who underwent CPB between January 1995 and January 2014 were matched with haemoglobin AA (HbAA) controls according to sex, age, weight and type of cardiac procedure. Thirty-three sickle-cell patients (21 HbAS, 7 HbSS and 5 HbSC) underwent CPB surgery using our institutional protocol. Sickle-cell patients and controls were similar according to the matching criteria. Preoperatively, haemoglobin SS (HbSS) and haemoglobin SC (HbSC) patients were anaemic (8.5 ± 1.4 vs 13.5 ± 1.9 g/dl; P <0.01 and 11.0 ± 0.6 vs 12.7 ± 0.9 g/dl; P = 0.01, respectively). Operative procedures included valve repair and replacement (12) as well as repair of congenital cardiac malformations (21). The duration of CPB and lowest CPB temperatures was similar for sickle-cell patients and controls. Systemic hypothermia (23.8-33.5°C), aortic cross-clamping, cold crystalloid antegrade cardioplegia and topical hypothermia were used in sickle-cell patients without complications. There was no acidosis, hypoxia or low cardiac output state. No mortality or important sickle-cell-related complications occurred. Although blood loss was similar between sickle-cell patients and controls, HbSS (unlike HbAS and HbSC) patients required more blood transfusion than controls (30.0 ± 13.3 vs 10.8 ± 14.2 ml/kg; P = 0.02) to counter haemodilution and replace blood loss. In-patient stay was similar for sickle-cell patients and controls. Perioperative exchange transfusion is not essential for a good outcome in sickle-cell patients undergoing CPB. A simple transfusion regimen to replace blood loss is safe in HbSS patients; blood transfusion requirements for HbSC and HbAS patients undergoing CPB are similar to those of matched HbAA controls. The use of systemic hypothermia during CPB does not increase sickle-cell-related complications. Cold crystalloid cardioplegia and topical hypothermia provide safe myocardial protection without the need for more sophisticated measures.

Simplified nonresectional leaflet remodeling mitral valve repair for degenerative mitral regurgitation

Simplified nonresectional leaflet remodeling mitral valve repair for degenerative mitral regurgitation

Assessment of pulmonary veins after atrio-pericardial anastomosis by cardiovascular magnetic resonance.

BackgroundThe atrio-pericardial anastomosis (APA) uses a pericardial pouch to create a large communication between the left atrium and the pulmonary venous contributaries in order to avoid direct suturing of the pulmonary veins during the repair of congenital cardiac malformations. Post-operative imaging is routinely performed by echocardiography but Cardiovascular Magnetic Resonance (CMR) offers excellent anatomical imaging and quantitative information about pulmonary blood flow. We sought to compare the diagnostic value of echocardiography and CMR for assessing pulmonary vein anatomy after the APA.MethodsThis retrospective study evaluated all consecutive patients between October 1998 and January 2010 after either a primary or secondary APA followed by post-repair CMR.ResultsOf 103 patients who had an APA, 31 patients had an analyzable CMR study. The average time to CMR was 24.6 ± 32.5 months post-repair. Echocardiographic findings were confirmed by CMR in 12 patients. There was incomplete imaging by echocardiography in 7 patients and underestimation of pulmonary vein restenosis in 12, when compared to CMR. In total, 19/31 patients (61%) from our cohort had significant stenosis following the APA as assessed by CMR. Our data suggest that at least 18% (19/103) of all patients had significant obstruction post-repair.ConclusionsEchocardiography incompletely imaged or underestimated the severity of obstruction in patients compared with CMR. Pulmonary vein stenosis remains a sizable complication after repair, even using the APA.

Utility of computed tomographic angiography in the pre-operative planning for initial and repeat congenital cardiovascular surgery

To investigate the utility of computed tomographic angiography as an adjunctive imaging modality before congenital cardiac surgery. We evaluated 33 patients who underwent a pre-operative computed tomographic angiogram. They were classified according to the anatomic site of repair. Post-operatively, the surgeon completed a questionnaire assessing the utility of the study. Computed tomographic angiography was found to be either "essential" or "very useful" for pre-operative planning in 94% of the patients. Specifically, the scan was consistently useful for procedures involving the aorta (14/15, 93%) or the pulmonary veins (4/4, 100%) and obviated pre-operative catheterisations in 14 patients (42%). Furthermore, when compared with other diagnostic groups, computed tomographic angiography determined the need for peripheral cannulation in patients undergoing re-operations (6/7; 86%, p = 0.02). Computed tomographic angiography was found to be useful in the pre-operative planning of virtually all patients undergoing repair of congenital cardiac malformations, regardless of diagnosis. Specifically, the studies were essential in select populations, such aortic arch or pulmonary vein repairs, and helped to determine cannulation sites for repeat operations while significantly reducing the need for invasive imaging.

Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease – the perspective of anaesthesia

The Congenital Cardiac Anesthesia Society was formed in 2005 by representatives from many of the busiest congenital cardiac surgical programs in North America and is now in the process of partnering with The Society of Thoracic Surgeons to create a joint congenital cardiac surgery and congenital cardiac anaesthesia database. Even the busiest of congenital cardiac programs have a low frequency of anaesthesia-related cardiac complications and deaths. One of the only mechanisms for accurately determining the incidence and outcomes of low frequency events is to aggregate large amounts of data from multiple sources. To that end, the Congenital Cardiac Anesthesia Society has joined with the Society of Thoracic Surgeons Congenital Database Task Force to incorporate anaesthesia-specific data points into their surgical registry, which is now the largest single reporting site for children and adults undergoing surgical repair of congenital cardiac malformations in North America. The Joint Congenital Cardiac Anesthesia Society--Society of Thoracic Surgeons Database will therefore become an optional module of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Initial data fields have been selected and are presented in this article. Efforts are ongoing to make this initiative a global project. Initial collaborative discussions have taken place about the possibility of linking this initiative with the European Association of Cardiothoracic Anesthesiologists. It is certainly possible and desirable that the planned anaesthesia module of The Society of Thoracic Surgeons Congenital Heart Database has an identical module in the congenital heart database of The European Association for Cardio-Thoracic Surgery and The European Congenital Heart Surgeons Association. This project should also ideally spread beyond North America and Europe. Efforts to involve Africa, Asia, Australia, and South America are necessary and already underway. The creation of a joint cardiac surgery and anaesthesia database is another step towards the ultimate goal of creating a database for congenital heart disease that spans both geographical and subspecialty boundaries.

Transoesophageal echocardiography in monitoring, guiding, and evaluating surgical repair of congenital cardiac malformations in children

To evaluate the role of transoesophageal echocardiography and problems related to safety during the surgical repair of congenital cardiac malformations in children. We examined the transoesophageal recordings made in 350 children with congenital cardiac diseases, aged from 2 months to 17 years and 9 months, with a median age of 2.7 years, tracings having been taken both before and after cardiopulmonary bypass. All patients had been scanned by transthoracic echocardiography before the operative procedures. Preoperative transoesophageal echocardiography added additional findings, or changed the diagnoses made using transthoracic echocardiography, in 33 cases (9.4%), among which the findings had therapeutic significance in 23 cases (6.6%) that altered the planned surgical procedures. Residual problems or sequels were detected by postoperative transoesophageal echocardiography in 57 cases (16.3%), with 13 patients (3.7%) requiring instant intervention or return to bypass for modifications of the surgical procedures. We encountered no severe complications due to the performance of transoesophageal echocardiography. Mild complications occurred in only 2 patients (0.6%). Transoesophageal echocardiography is a useful tool with which to determine the strategies for treatment in the perioperative period, and to improve the quality of surgical procedures in children with congenital cardiac diseases. Complications were few, but still deserved careful attention to detail.

Outcome after surgical repair of congenital cardiac malformations at school age

To explore the long-term physical, educational, behavioural, and emotional outcome of patients undergoing surgical correction of congenital cardiac disease at school age, and to investigate the relation, if any, between the outcome and comorbidity, age and sex, and level of complexity of the cardiac surgery. Information was obtained concerning 101 patients who underwent open-heart surgery for correction of congenital cardiac malformations between 1992 and 2000 whilst aged from 6 to 16 years. The patients, and their parents, completed the questionnaire "Outcome of congenital heart disease and surgery", the RAND 36-Item Health Survey, and the Child Behaviour Checklist/Youth Self-Report/Young Adult Self-Report. Of the patients, 26% had comorbidity. Of those without comorbidity, 39% had frequent physical complaints, and 28% experienced limitations due to the cardiac disease. Nevertheless, the patients reported a good subjective state of health, and did not report any behavioural or emotional problems. Patients did show academic difficulties. They had received special education more frequently than their healthy peers, and many had needed to repeat a grade, or had received remedial teaching. Consequently, the educational level of patients was lower than that of their healthy peers. Patients with comorbidity, female patients, and patients who underwent complex surgery, seemed to be most at risk for physical, behavioural, and emotional problems. It is necessary to distinguish between physical state and its appraisal, and clinicians should be aware of this. Further research is needed to find out the cause and nature of the academic difficulties. Groups of patients at risk should be followed closely to enable early interventions.

Experience with intraoperative ultrasound in paediatric cardiac surgery

Intraoperative ultrasound was introduced to evaluate the adequacy of repair after surgical repair of congenital cardiac malformations. Our purpose was to review the evolution of this technique at our centre. We evaluated all intraoperative ultrasound studies undertaken between 1997 and 2002, reviewing the data from 1997 through 2001 retrospectively, but undertaking a prospective audit of studies undertaken from 2002 onwards. In all, we carried out a total number of 639 intraoperative ultrasound studies, from a possible 2737 cardiac operations (23.3%), using the epicardial approach in 580 (90.7%), and transoesophageal ultrasound in the other 59 patients (9.3%). Median age was 0.6 years, with an interquartile range from 0.06 to 3.6 years. The findings obtained using intraoperative ultrasound influenced the surgical management in 63 of the 639 patients (9.9%), suggesting the need for additional surgery in 26, adjustment of the band placed round the pulmonary trunk in 16, preoperative assessment of the cardiac malformation in 5, and confirming the need for prolonged support with cardiopulmonary bypass for impaired ventricular function in 16. There were 18 early reoperations, 5 of which may have been predicted by intraoperative ultrasound. Of the 183 studies reviewed prospectively in 2002, it was not possible to obtain the complete range of views in 8 (4.4%), while in 27 patients (14.7%), the postoperative findings using transthoracic interrogation differed from the findings obtained immediately following bypass. Intraoperative ultrasound is an important technique for monitoring the results of complex congenital cardiac surgery. The immediate recognition of significant lesions, together with multidisciplinary discussion, allows for improved management and prevention of early surgical reintervention.

Assessing the brain using near-infrared spectroscopy during postoperative ventricular circulatory support

Due to many innovative techniques and technologies, the overall rate of survival for infants undergoing surgical repair of congenital cardiac malformations is excellent. Despite these excellent outcomes, neonates with hypoplasia of the left heart represent a very unique population, and pose difficult challenges for the entire team involved in care. Despite some centers reporting outstanding outcomes, overall rate of survival rate is reported at only 53 percent,1 and survivors often show neurodevelopmental deficits. Many attempts have been made to identify predictors of neurologic outcomes, with heavy concentration around the pre- and peri-operative periods. Little attention has been paid, however, to assessing how attempts at balancing the systemic and pulmonary circulation affect neurodevelopmental outcomes. In the past, we have reported excellent rates of survival with use of routine postoperative circulatory support to augment cardiac output.2,3 This strategy, nonetheless, is still widely debated. It seems plausible that, by augmenting cardiac output in the immediate postoperative period, cerebral perfusion and oxygenation can be optimized, thereby improving neurologic outcomes. In this review, we describe our experience to date in assessing neurological outcomes, and discuss whether outcomes are associated with cerebral saturation and extraction of oxygen during the period of postoperative circulatory assist.

The role of vasopressin in treating systemic inflammatory syndrome complicated by right ventricular failure

Severe right ventricular failure complicated a postoperative systemic inflammatory response in a 33-year-old woman after surgical repair of congenital cardiac malformations. Volume loading, and high doses of catecholamines, failed to produce improvement, but treatment with vasopressin improved all haemodynamic parameters, and also allowed reduction of the other inotropes. After 10 days, the patient was discharged in stable condition from the intensive care unit.

Parental decision-making in congenital heart disease

To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. A combined quantitative-qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

Thoracoscopic closure of the patent arterial duct

Video assisted thoracoscopic surgery for closure of the persistently patent arterial duct is an effective answer to the modern effort of seeking repair of congenital cardiac malformations with minimally invasive techniques. Between June, 1994, and December, 2002, 150 consecutive patients with an echocardiographic diagnosis of isolated patency of the arterial duct were referred to our Institution for video assisted thoracoscopic closure. The median age at the time of operation was 45 months, with a range from 3 to 161 months, and mean weight was 18 kg, with a range from 4.2 to 73 kg. Video assisted closure was possible in 139 patients (93%). The mean operative time was 37 min, with a range from 14 to 89 min. In the remaining 11 patients (7%), seen early in our experience, we converted to a mini-thoracotomy to achieve closure. In no instance did we encounter major haemorrhage requiring blood transfusion, and there were no hospital deaths. The mean period of stay in hospital was less than 24 h in 61 patients, less than 48 h in 88 patients seen earlier in our experience, and 21 days in 1 patient because of a persistent chylothorax. Other complications included palsy of the left recurrent laryngeal nerve in 1 patient, with partial regression at 6 months follow-up. We conclude that closure of the arterial duct by video-assisted thoroscopy combines the advantages of safe and complete extraluminal occlusion with an excellent clinical and aesthetic result, minimal postoperative pain, and short stay in hospital at low cost.

Tratamiento mediante hipotermia de la taquicardia ectópica de la unión tras cirugía cardíaca infantil

Junctional ectopic tachycardia (JET) is a complication of the repair of congenital cardiac malformations that responds poorly to conventional treatment. We report our experience with the use of moderate hypothermia in its management. Twelve infants with postoperative JET treated with hypothermia were reviewed. The mean interval between the diagnosis of JET and initiation of hypothermia was 1.5 0.5 hours. In the first 24 hours of hypothermia, central temperature and heart rate decreased significantly. Arterial pressure and diuresis tended to increase and central venous pressure tended to decrease. No direct adverse events occurred. All the patients but one survived and are alive and free of neurological deficits after 15 12 months.

Results of biventricular repair of congenital cardiac malformations: definitive corrective surgery?

Congenital cardiac malformations are usually corrected in the neonatal period or in early infancy. Corrective surgery may not always be definitive, especially in complex malformations. Long-term morbidity is influenced by reoperations and their risk. This study analyzes our single-center experience over more than 20 years in a selected group of patients. Data were gathered, with special focus on causes and incidence for reoperations, respectively. Freedom from reoperation after 5, 10, and 15 years for each cardiac malformation was determined. The numbers describe in the following order patient years (y), number of patients (n), and freedom from reoperation at follow-up interval (%), respectively: atrial septal defect (15,864y, n=1198, 99+/-0/99+/-0/99+/-0), partial atrioventricular septal defect (2506y, n=234, 95+/-2/93+/-2/93+/-2), total anomalous pulmonary venous connection (742y, n=141, 93+/-1/91+/-0/91+/-0), complete atrioventricular septal defect (1715y, n=377, 81+/-3/76+/-3/72+/-4), tetralogy of Fallot <1 year (1503y, n=197, 94+/-4/85+/-1/74+/-3), transposition of great arteries (1459y, n=375, 88+/-2/83+/-4/73+/-7), interrupted aortic arch (IAA) (481y, n=98, 63+/-6/52+/-7/45+/-8), common arterial trunk (CAT) (599y, n=109, 64+/-6/24+/-6/11+/-5). In most congenital malformations surgical correction is definitive and the rate of reoperations is low. In complex anomalies, such as CAT and IAA, reoperations at long-term are more common. Analysis of such results and recognition of a sometimes inevitable operative morbidity helps to predict long-term outcome and influences the follow-up.

Anomalous course of the left brachiocephalic vein

An anomalous course of the left brachiocephalic vein beneath the aortic arch was identified in 7 patients undergoing surgical repair of congenital cardiac malformations. Six of these patients had the morphologic features of tetralogy of Fallot including severe obstruction to the right ventricular outflow tract and a right aortic arch. A review of the literature reveals this to be an uncommon anomaly. When it is present, however, it is frequently associated with a ventricular septal defect, obstruction of the right ventricular outflow, and aortic arch anomalies. This anomaly can be documented by echocardiography, at cardiac catheterization, or intraoperatively. The presence of a subaortic left brachiocephalic vein may have implications for the conduct of surgical procedures, especially in the setting of tetralogy of Fallot.

Nitroprusside abolishes the deleterious effects of surface cooling–induced hypothermia on immature pigs with ventricular septal defects

The efficacy of systemic hypothermia in combination with cardiopulmonary bypass for the repair of congenital cardiac malformations is established. Surface cooling in infants with ventricular septal defects as a prebypass adjunct has been associated with visceral ischemic complications. Surface cooling in infant pigs with ventricular septal defects results in increased systemic vascular resistance and unchanged pulmonary vascular resistance with increased left-to-right shunting and a maldistribution of blood flow away from the viscera and kidneys. This study was to determine whether nitroprusside ameliorates the deleterious effects of increased systemic vascular resistance during hypothermia in infant pigs with ventricular septal defects. Nine neonatal pigs (4 weeks, 4 kg) had experimental ventricular septal defects resulting in a 2.3 +/- 0.3: 1 shunt. Systemic and pulmonary vascular resistances were determined by the flow probe and microsphere techniques. Systemic and pulmonary blood flow distributions were measured by the microsphere technique. Left-to-right shunt ratios were determined by oximetry, flow probe, and microsphere. The pigs were cooled to 28 degrees C and measurements were made at 37 degrees, 32 degrees, and 28 degrees C. We found that nitroprusside abolishes the deleterious effects of surface cooling by blocking the rise of systemic vascular resistance without significant changes in aortic pressure. As a result, the animals maintained control pulmonary-to-systemic flow levels and maintained control levels of oxygen delivery while showing a decline in oxygen consumption. Additionally, the vasodilatory effect allowed the animals to cool twice as fast as animals without nitroprusside. Regional blood flow distribution as percent cardiac output and absolute tissue flow were protected during surface cooling. This technique may have a role in cardiac operations on infants with left-to-right shunts.

Take-down of cava-pulmonary artery anastomosis (Glenn) during repair of congenital cardiac malformations : A. D. Pacifico and J. W. Kirklin. J Thorac Cardiovasc Surg 70:272–277 (August), 1975

Take-down of cava-pulmonary artery anastomosis (Glenn) during repair of congenital cardiac malformations : A. D. Pacifico and J. W. Kirklin. J Thorac Cardiovasc Surg 70:272–277 (August), 1975

Take-down of cava-pulmonary artery anastomosis (Glenn) during repair of congenital cardiac malformations: Report of 5 cases

The dismantling of previously made anastomses between the superior vena cava and right pulmonary artery (Glenn shunts) and reconstruction of superior vena cava-right artial continuity have been accomplished in 5 patients at the time of intracardiac repair of congenital heart defects. All patients survived the hospitalization period, but 2 died late postoperatively of causes apparently not related to the cava-atrial reconstruction. Experience with these 5 patients and the intraoperative techniques employed are presented.