Video-assisted endoscopy for congenital heart repair.

Endoscopic imaging techniques can be used to enhance visualization of and access to remote intracardiac structures to improve congenital heart repairs. Database storage of these images builds a foundation for retrospective analysis of surgical failures and clinical correlations with other imaging techniques. The images also function as an educational tool for patients, families, and the cardiac team. Combining cardiac endoscopic imaging with interventional catheterization techniques has created a group of hybrid procedures, extending the capabilities of both the surgeon and the interventional cardiologist. This synergy has the potential to decrease therapeutic trauma.

Reducing the trauma of congenital heart surgery.

Techniques to reduce surgical trauma for congenital heart repairs continue to evolve in tandem with advances in the more conventional protection strategies. The concept of chest wall protection has been increasingly accepted as an important standard for congenital heart surgeons, as long as neuroprotection, myocardial protection, and operative precision are not adversely affected. Unfortunately, it is difficult to measure chest wall trauma, making it difficult to evaluate the efficacy of minimally invasive techniques. Efforts to reduce surgical trauma are advancing on multiple fronts. Many extracardiac repairs are being performed thoracoscopically, with excellent results. Intracardiac procedures can be performed through smaller incisions. Full sternotomy is giving way to a variety of sternal-sparing incisions for open-heart repairs, usually using direct visualization. Operative strategies to avoid or minimize the effects of cardiopulmonary bypass have emerged. And recently, hybrid procedures, utilizing a combination of transcatheter and minimally invasive techniques, are creating new therapeutic options for the congenital heart team.

Anomalous origin of innominate artery from right pulmonary artery in DiGeorge Syndrome.

We experienced a case of anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery). This patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome, who was diagnosed with perimembranous ventricular septal defect, atrial septal defect, and patent ductus arteriosus. This type of anomaly is exceedingly rare.

Assisted venous drainage cardiopulmonary bypass in congenital heart surgery.

BACKGROUND: A novel active venous drainage perfusion circuit was designed to achieve effective venous return through small venous cannulas. The efficacy and safety of this new system was investigated and compared with a conventional gravity drainage system. METHODS: Four hundred consecutive patients undergoing open heart repair of congenital heart lesions by one surgeon were studied. The first 200 patients were supported by gravity drainage and the next 200 patients were supported by assisted venous drainage. No patient in the time period was excluded from the study. RESULTS: The two groups did not differ significantly in weight, bypass time, or cross-clamp time. Priming volumes were less in the assisted group than in the gravity group (576+/-232 mL versus 693+/-221 mL, p < 0.001). Venous cannula size was smaller in the assisted group when compared with the gravity group (33.2F+/-7.4F versus 38.5F+/-7.1F, p < 0.001). There was a trend to lower operative mortality in the assisted drainage group (5 of 200, 2.5% versus 11 of 200, 5.5%; p = 0.10). Hospital stay and pulmonary, infectious, and neurologic complications were comparable in both groups. Cardiac complications were less common in the assisted group than in gravity group (22 of 200, 11% versus 38 of 200, 19%; p = 0.017). Hematologic complications were less common in the assisted group than the gravity group (6 of 200, 3% versus 19 of 200, 9.5%; p < 0.01). CONCLUSIONS: These findings suggest that assisted venous drainage is safe in congenital heart operations and facilitates the use of smaller venous cannulas.

Endovascular stent graft treatment of a pulmonary artery pseudoaneurysm.

A 5-year-old girl with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and multiple pulmonary artery reconstructive procedures presented with an enlarging pulmonary artery pseudoaneurysm. A previous attempt to occlude the aneurysm was unsuccessful and the aneurysm continued to enlarge. We describe the percutaneous placement of an endovascular stent graft to occlude the aneurysm. This novel use of a covered graft effectively treated a potentially lethal problem without reoperative thoracotomy or sternotomy.

Reducing the trauma of congenital heart surgery.

While describing the circulatory system in De Moto Cordis, in 1628, William Harvey developed precepts for investigation, which could be modified slightly to guide the adoption of new technology and technique in the twenty-first century. Harvey might suggest (1) careful and accurate observation and description of a new technique, (2) a tentative explanation of how the technique improves on existing techniques, (3) a controlled testing of the hypothesis, and (4) conclusions based on the results of the experiments. Also, he might admonish surgery today, with its massively enhanced capabilities for information management, to rigorously test the validity of these conclusions with quantitative reasoning. In the future, precise measurement of the "trauma" of surgery, or even an individual surgeon, may be possible, and the long-term impact of a chest wall incision on a patient's self-esteem may be predictable. Absent such objective measures, justifications for "minimally invasive" deviations from conventional technique in surgery for CHD lack substance. Morbidity, mortality, and physiological endpoints will continue to form the foundation for therapeutic plans; however, the potential for emerging technology to reduce the trauma of these plans remains tantalizing.

Video-assisted cardioscopy for intraventricular repair in congenital heart disease.

BACKGROUND: Video-assisted thoracoscopic surgical techniques have been widely adopted as a means to reduce surgical trauma. By adapting pediatric thoracoscopic instrumentation, we have developed a technique for video-assisted cardioscopy (VAC). We report our experience and describe the technical feasibility of VAC. METHODS: Since June 1995, 409 consecutive patients underwent 431 intracardiac procedures (ventricular septal defect, 150; tetralogy of Fallot or double outlet right ventricle, 101; atrioventricular canal, 52; subaortic stenosis, 43; valve repair, 50; Rastelli procedure, 12; Konno or Ross Konno operation, 11; and miscellaneous, 12) using VAC at Miami Children's Hospital. Using a prospective database, we tracked outcomes and operative events to delineate the usefulness and efficacy of this technique. RESULTS: VAC provided clear and precise imaging of small or remote intracardiac structures during repair of congenital heart defects without technical complications. Procedure times and aortic cross-clamp times using VAC were not prolonged. Intraoperative images were collected for every operation, documenting each patient's cardiac anatomy before and after repair. Surgery through small incisions was facilitated. Operative mortality was 1.2% (5 of 409), and no patient required reoperation before discharge. At a mean follow-up interval of 22 months, the incidence of reoperation for residual or recurrent lesions was 1.2% (5 of 404). CONCLUSIONS: Our experience demonstrates the technical feasibility and clinical utility of routine endoscopic imaging during open heart surgery for congenital heart repair.

Rapid cardiopulmonary support for children with complex congenital heart disease.

BACKGROUND: Extracorporeal membrane oxygenation has limitations in children with congenital heart disease (prolonged setup times, increased postoperative blood loss, and difficulty during transport). We developed a miniaturized cardiopulmonary support circuit to address these limitations. PATIENTS AND METHODS: The cardiopulmonary support system includes a preassembled, completely heparin-coated circuit, a BP-50 Bio-Medicus centrifugal pump, a Minimax plus membrane oxygenator, a Bio-Medicus flow probe, and a Bio-trend hematocrit/oxygen saturation monitor. Short tubing length permits a 250-mL bloodless prime in less than 5 minutes. From 1995 to 1997, 23 children with congenital heart disease were supported with this technique. RESULTS: Overall survival to discharge was 48% (11 of 23 patients). Survival to discharge was 80% (4 of 5) in the preoperative support group, 20% (1 of 5) in the postoperative failure to wean from cardiopulmonary bypass group, 44% (4 of 9) in the group placed on support postoperatively after transfer to the intensive care unit, and 50% (2 of 4 patients) in the nonoperative group. Neonatal cardiopulmonary support survival to discharge was 46% (6 of 13 patients). CONCLUSIONS: This pediatric cardiopulmonary support system is safe and effective. Advantages over conventional extracorporeal membrane oxygenation include rapid setup time, decreased postoperative blood loss, and simplified transport.

Outcome of cardiopulmonary resuscitation in a pediatric cardiac intensive care unit.

OBJECTIVE: To determine the eventual outcome of children with heart disease who had cardiopulmonary resuscitation (CPR) in a specialized pediatric cardiac intensive care unit (CICU), and to define the influence of any prearrest variables on the outcome. DESIGN: A retrospective review of patients' medical records. SETTING: A pediatric CICU of a tertiary pediatric teaching hospital. PATIENTS AND METHODS: Patients were all children who presented with cardiopulmonary arrest and who were administered CPR in the pediatric CICU between June 1995 and June 1997. Prearrest variables such as age, diagnosis, prior cardiac surgery, and inotropic support with epinephrine, as well as cause of arrest, were evaluated. MEASUREMENTS AND MAIN RESULTS: Thirty-two patients, ranging in age from 1 day to 21 yrs (median, 1 month), satisfied criteria for inclusion in the study group. These 32 patients had a total of 38 episodes of cardiopulmonary arrest. Twenty-five of these patients (78%) had cardiac surgery before arrest. Inotropic support with continuous infusion of epinephrine was being administered at the time of arrest in 18 of 38 (47%) arrests. These prearrest variables did not influence outcome of CPR. Of the 38 episodes of CPR, 24 episodes (63%) were successful, with 20 episodes resulting in return of spontaneous circulation and four patients being successfully placed on mechanical cardiopulmonary support. Fourteen children, including all four patients who were rescued with mechanical cardiopulmonary support, survived to discharge. At 6-month follow-up, 11 patients were still alive, with three having neurologic impairment. CONCLUSIONS: After cardiopulmonary resuscitation in this pediatric CICU, the rate of success was 63% and the rate of survival was 42%. Prior cardiac surgery and use of epinephrine before arrest did not influence the outcome of CPR. The availability of effective mechanical cardiopulmonary support can improve the outcome of CPR.

Congenital Heart Surgery Nomenclature and Database Project: atrial septal defect.

The extant nomenclature for atrial septal defect (ASD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect.

The extant nomenclature for ventricular septal defect (VSD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.

The extant nomenclature for atrioventricular (AV) canal/atrioventricular septal defect is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The three general categories are: partial AV canal (ostium primum defect), transitional (intermediate) AV canal, and complete AV canal. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window.

The extant nomenclature for aortopulmonary window (AP window) and pulmonary artery origin from ascending aorta (hemitruncus) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Minimally invasive resection of congenital subaortic stenosis.

Fifteen consecutive patients with membranous subaortic stenosis underwent resection by a minimal-access approach through a partial upper sternotomy using a cardioscope. There were no operative deaths and no postoperative complications. Twelve patients (80%) were extubated in the operating room. The mean hospital stay was 3.1 days, and 3 patients (20%) needed blood products. Our experience demonstrates that this modified approach is a safe and effective surgical option for resection of subaortic stenosis.

The Ross operation in a Jehovah's Witness: a paradigm for heart surgery in children without transfusion.

A 3-year-old 18 kg male child of the Jehovah's Witness faith presented with severe aortic regurgitation. A successful Ross procedure was performed using a pulmonary autograft, without the use of blood or blood product transfusion. Blood conservation strategy included: (1) preoperative treatment with recombinant human erythropoietin; (2) intraoperative strategies, including technical modifications to the Ross procedure, and the prophylactic use of fibrin glue; (3) utilization of a heparin-bonded cardiopulmonary bypass circuit and assisted venous drainage; and 4) the use of prebypass phlebotomy, cell-saving device and autotransfusion. The patient was discharged home on postoperative day 7 with a hemoglobin level of 11.9.

Heparin-coated cardiopulmonary bypass circuit: clinical effects in pediatric cardiac surgery.

OBJECTIVES: Heparin-coated cardiopulmonary bypass (CPB) circuits have been reported to reduce complement activation and the inflammatory response associated with CPB. We retrospectively compared patients utilizing heparin-coated perfusion circuits with those using noncoated circuits to determine the clinical effects of the different circuits in pediatric cardiac surgery. METHODS: Between July 1995 and July 1997, 203 patients weighing < 10 kg underwent cardiac surgery, 153 patients using heparin-coated bypass circuits and 50 patients using noncoated circuits. The 50 patients operated on with the noncoated circuit (Group N) were matched to 100 patients operated on with coated circuits (Group H) in age, weight, and type of procedure. Urine output during bypass, blood products used after bypass, postoperative ventilation days, hospital stay, morbidity, and mortality were compared between these groups. RESULTS: Body weight, perfusion time, and procedure time were not different between the two groups. Urine output during bypass was notably greater in Group H than in Group N (11.3 +/- 10.5 mL/kg per hour vs 4.8 +/- 3.1 mL/kg per hour, respectively, p < 0.0001). Postoperative mechanical ventilation markedly decreased in Group H (Group H vs N = 2.8 +/- 2.7 days vs 5.1 +/- 7.5 days, respectively, p < 0.05). Red blood cell usage, hospital stay, morbidity, and mortality were not statistically different, although there was a tendency toward decreased transfusion of red cell and platelets in Group H (Group H vs N = 61.2 +/- 121.1 mL/kg vs 102.0 +/- 176.7 mL/kg, respectively, in red cell, p = 0.15; and Group H vs N = 7.9 +/- 13.7 mL/kg vs 13.2 +/- 24.5 mL/kg, respectively, in platelets, p = 0.16). CONCLUSIONS: Patients operated on with the use of heparin-coated circuits had increased urine output during bypass and required less time postoperatively on the ventilator. These results suggest a reduction in the acute inflammatory response, capillary leakage, and overall systemic edema. We now routinely use coated circuits on all pediatric pump cases.

Tracheal allograft reconstruction: the total North American and worldwide pediatric experiences.

BACKGROUND: We reviewed both the North American and the total worldwide pediatric experience with tracheal allograft reconstruction as treatment for patients with long segment and recurrent tracheal stenosis. METHODS: The stenosed tracheal segment is opened to widely patent segments. The anterior cartilage is resected and the posterior trachealis muscle or tracheal wall remains. A temporary silastic intraluminal stent is placed and absorbable sutures secure the chemically preserved cadaveric trachea. After initial success with this technique in Europe, several North American centers have now performed the procedure. The cumulative North American experience includes 6 patients (3 adults and 3 children). Worldwide, more than 100 adults and 31 children, aged 5 months to 18 years, with severe long segment tracheal stenosis have undergone tracheal allograft reconstruction. RESULTS: In North America, 5 of 6 patients have survived, with one early death due to bleeding from a tracheal-innominate artery fistula in a previously irradiated neck. Worldwide, 26 children survived (26 of 31 = 84%) with follow-up from 5 months to 14 years. Only 1 of 26 pediatric survivors (1 of 26 = 3.8%) had a tracheostomy. CONCLUSIONS: Tracheal allograft reconstruction demonstrates encouraging short- to medium-term results for patients with complex tracheal stenosis. Allograft luminal epithelialization supports the expectation of good long-term results.

Video-assisted thoracoscopic surgery for patent ductus arteriosus in low birth weight neonates and infants.

BACKGROUND: Video-assisted thoracoscopic surgery (VATS) has been assuming an expanded role in the management of cardiothoracic disease. As instrumentation and experience increase, VATS is being applied to treat smaller patients. We report our experience with 34 low birth weight infants undergoing VATS interruption of patent ductus arteriosus (PDA). METHODS: VATS allows PDA interruption without the muscle cutting or rib spreading of a standard thoracotomy. Four small, 3-mm incisions are made along the line of a potential thoracotomy incision. Ports placed through these incisions admit endoscopic instruments, a camera, and a vascular clip applier. RESULTS: Median age at surgery was 15.5 days (range: 1-44 days). Median weight at surgery was 930 g (range: 575-2500 g). Twenty patients weighed <1 kg, and 13 weighed <750 g. All patients had congestive heart failure and had either failed indomethacin therapy or had contraindications to indomethacin. Median surgical time was 60 minutes (range: 31-171 minutes). Echocardiography documented elimination of ductal flow in all patients. Operative mortality was zero. Four patients (4/34 = 12%) required conversion to open thoracotomy: 1 because of difficult exposure, 1 because of pulmonary dysfunction and anasarca, 1 because of a large 1-cm duct, and 1 because of coagulopathy and poor pulmonary compliance. Two patients died before discharge: 1 patient (surgical weight: 605 g) died on postoperative day 2 because of intracranial hemorrhage, and 1 patient (surgical weight: 1725 g) died on postoperative day 88 because of multiple system organ failure. Follow-up has demonstrated no PDA murmur in any patient, but echocardiography revealed trace ductal flow in 2 patients. CONCLUSIONS: VATS offers a minimally traumatic, safe, and effective technique for PDA interruption in low birth weight neonates and infants.

Adverse hemodynamic effects observed with inhaled nitric oxide after surgical repair of total anomalous pulmonary venous return.

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.

Minimally invasive pediatric cardiac surgery.

Minimally invasive cardiac surgery has evolved in response to the intrinsic irony facing cardiac surgeons: that we must injure our patients to treat them. In recent years, advances in fiberoptic imaging technology, applied to other surgical specialties, suggested the possibility that cardiac surgery might also be performed endoscopically. The anatomic and spatial constraints of pediatric cardiac surgery, and its dependence on extreme levels of speed, precision, and three-dimensional perception, made the application of remote, two-dimensional operating systems seem impossible, or at least imprudent in this special group of patients. Despite these limitations, however, applications of video-assisted endoscopic surgical techniques have been demonstrated to allow the safe and effective performance of an expanding range of operative procedures in congenital heart surgery. The guided development of new technology will accelerate this process in the coming years.