The modified Senning operation for cavopulmonary connection with autologous tissue.

UNLABELLED: A modified Senning technique was used for intraatrial channeling of the systemic venous blood into the pulmonary arteries to create a Fontan circulation in 26 children, six with tricuspid atresia and 20 with complex congenital heart disease. In this technique a flap of atrial free wall tissue is used to create an atrial tunnel without artificial material. Eight patients had subaortic stenosis and required a Damus-Kay-Stansel procedure, in addition. Early mortality was two of 26 and late mortality one of 26. Pleural effusion was encountered in 17 of 26 patients, of whom four had a pericardial effusion, in addition. One patient required pacemaker implantation for complete atrioventricular block. Follow-up ranged from 2 months to 5 years. In this period the ability level index rose by one level. No thrombi were encountered in the right atrium/cavopulmonary tunnel. One patient required antiarrhythmic medication. Protein-losing enteropathy was diagnosed in one patient. CONCLUSION: This modified Senning technique has the advantage of avoiding the use of prosthetic material in the creation of a Fontan circulation and the potential for fewer long-term complications.

Morphology of pulmonary atresia with intact ventricular septum in patients dying after operation.

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.

Intraoperative flow measurement of native coronary artery can help decision making before CABG.

We report the case of a patient with three-vessel coronary artery disease whose right coronary artery had been stented at the time of the diagnostic procedure. He had recurrent angina 12 days later and was transferred for urgent coronary artery bypass grafting. No repeat coronary angiography was performed. In the operating room, the flow on the native right coronary artery was determined with an ultrasonic flow probe.

Complication of intra-aortic balloon pumping in a pediatric patient.

A serious complication was seen following insertion of an intra-aortic balloon pump in the ascending aorta in a pediatric patient. The catheter initially coursed to the left subclavian artery from which it folded upon before finding its way in the descending aorta. We recommend that a suitably curved J-tip guidewire be used to guide the IABP balloons distal to the aortic arch into the descending aorta, so as to avoid potentially fatal complications.

Mid-term results of the modified Senning operation for cavopulmonary connection with autologous tissue.

UNLABELLED: The aim of this study was to determine the results and mid-term outcome of a modified Senning technique using autologous tissue for total cavopulmonary connection. The study involved 31 children, 8 with tricuspid atresia and 23 with complex congenital heart disease. In this operation, a flap of autologous atrial free wall tissue was used to tunnel inferior vena caval blood to the pulmonary arteries. An additional Damus-Kay-Stansel operation was required in 9 patients with subaortic obstruction. RESULTS: the early mortality rate was 16% (5 out of 31 patients) and there were four late deaths. COMPLICATIONS: Pleural effusions were encountered in 17 patients, of whom 4 had a concomitant pericardial effusion. Diaphragmatic paralysis was diagnosed in five patients, one of whom underwent surgical plication. Median hospital stay was 26 days. The 1- to 5-year actuarial survival was 68.6%. Follow-up ranged from 10 months to 7.1 years, mean 3.2 years. A serious atrial arrhythmia was diagnosed in one patient and another one died, possibly from rhythm disorders. Exercise tolerance and quality of life has improved in all but one of the survivors. Although follow-up is short, we have thus far witnessed a low incidence of hemodynamic and rhythm disturbances with this modification of the cavopulmonary connection.

Bi-directional cavopulmonary shunt: is accessory pulsatile flow, good or bad?

OBJECTIVE: Evaluation of the effect and long-term outcome of accessory pulsatile blood flow versus classical bi-directional cavopulmonary connection (BCPC). METHODS: Retrospective review of the medical and surgical records. RESULTS: Two-hundred and five patients (119 boys, 86 girls) underwent BCPC from 1990 to 1996. Accessory pulsatile flow was present in 68%, flow being maintained through the pulmonary trunc in 46%, systemic-to-pulmonary artery shunt in 13% and mixed in 7%, or patent ductus arteriosus in 2%. Patients with accessory pulsatile flow had lower hospital mortality (3% versus 5%), while mean pulmonary artery pressure (14.1 versus 12.6 mmHg P = 0.050) and increase of oxygen saturation (12.4 versus 8.7, P = 0.034) were significantly higher. The period of artificial ventilation (1.9 day) and ICU stay (6 days) did not differ for both groups. Late mortality was higher following accessory pulsatile flow (6% versus 1%). At late follow-up patients with accessory pulsatile flow had significantly higher oxygen saturation (mean 85 +/- 4%, versus 79 +/- 4%; P < or = 0.005). If subsequent completion of Fontan is considered the optimal palliation and subsequent systemic to pulmonary artery shunt, arteriovenous fistula and transplantation is considered a failure, patients with accessory pulsatile flow had significantly more and earlier completion of the Fontan procedure (mean 1.7 +/- 2.4 years, versus 2.7 +/- 4.4 years; P = 0.008). Survival is not influenced by age at bi-directional cavopulmonary shunt surgery, left or right functional ventricular anatomy or previous palliative surgery. One patient with accessory pulsatile flow developed systemic-to-pulmonary collateral's eventually requiring lobectomy. CONCLUSION: Despite two different initial palliative techniques the outcome was not significantly different. Accessory pulsatile blood flow appeared not to be a contra-indication for a completion Fontan procedure. Moreover, the data suggest that after accessory pulsatile flow can safely be performed, at late follow-up oxygen saturation is higher, while, significantly more and earlier completion of Fontan occurred. Age at bi-directional cavopulmonary shunt, basic left or right ventricular anatomy or previous palliative surgery did not influence survival.

Cardiac surgery by transxiphoid approach without sternotomy.

OBJECTIVE: In a attempt to avoid the potential drawbacks associated with sternotomy coupled with a desire for a smaller scar led us to investigate the transxiphoid approach without sternotomy. We present our preliminary experience and a comparison between the sternal and thoracic approaches. METHODS: From June 1996, at the Institut Cardiovasculaire Paris Sud, Massy, France (ICPS) and the Heart Institute, Sao Paulo, Brazil (HI) the transxiphoid approach was adopted for the correction of selected congenital cardiac defects. The xiphoid was resected through a 6 cm long vertical skin incision. With a special retractor the sternum was elevated cephalad and anteriorly. Closure of the defect was performed in the conventional manner. Twenty-six patients; 17 boys and 9 girls were entered into the study from representing 19 atrial septal defects (ASDs), 4 ventricular septal defects (VSDs) and 3 partial atrio ventricular septal defect (AVSDs). In addition at ICPS the transxiphoid approach for correction of ASD was compared to the thoracic and sternal approaches performed in the same period. RESULTS: Both the aortic cross clamp time as well as the duration of extracorporeal circulation were increased when compared to either standard sternotomy or thoracotomy approaches. There were no differences within the groups when comparing body surface area, amount of chest drainage or length of either ICU or hospital stay. However the patients in the transxiphoid group showed less pain and respiratory discomfort. CONCLUSION: Our initial experience with the transxiphoid approach without sternotomy confirms that it is a promising technique that can be considered an alternative to conventional sternotomy. The access is adequate for surgical procedures performed through a right atriotomy. The advantages include a better cosmetic scar, less surgical trauma, minimal respiratory discomfort and a potentially lower risk of infection. However cardiopulmonary bypass and cross clamp times are increased. There were no complications, and patient satisfaction was high.

Post cardiac surgery phrenic nerve palsy: value of plication and potential for recovery.

OBJECTIVES: Evaluation of an aggressive policy for the treatment of phrenic nerve palsy (PNP), following cardiac operations, with emphasis on early diaphragmatic plication. Attention was given to the incidence and predisposing factors for PNP and the potential for recovery following plication. METHODS: From 1 June 1991 to 1 January 1996 we prospectively screened patients for PNP following cardiac surgery. The diagnosis was suspected if difficulty was experienced in weaning the child from the ventilator. If abnormal elevation of the hemidiaphragm was present diaphragmatic plication was performed. Echocardiography was used to assess subsequent return of diaphragmatic function. RESULTS: Seventeen children (nine boys, eight girls), out of 867 (1.9%) children younger than 16 years of age, undergoing cardiac operations were found to have PNP. The mean age was 66 days (range 1-17 months) with 16 patients below 1 year out of a total of 285 patients (incidence 5.6%) and one patient 17 months old. The incidence following open procedures was 11/190, following closed procedures 2/95 and following reoperation 4/83. PNP was diagnosed from 2 to 44 days (mean 14 days) following surgery. It was present on the right side in seven cases, the left in nine and was bilateral in one patient. Two patients were extubated at the time of diagnosis, one patient could be extubated shortly thereafter. Fourteen children underwent diaphragmatic plication, at a median 5 days post diagnosis. Extubation was possible 1-60 days (mean 4 days) after plication. Mean follow-up was 19 +/- 5 months. Subsequent recovery of diaphragmatic movement was documented in seven (41%) children. Time to recovery following plication was 16 months, without plication 38 months. CONCLUSION: Prospective screening for PNP revealed an incidence in children younger than 1 year of 6%. Early plication substantially reduces the duration of ventilation, with its associated reduced morbidity and ICU stay.

Diagnosis and management of the coronary-subclavian steal syndrome.

A patient is presented with recurrent angina due to a coronary-subclavian steal syndrome 3 years after left internal mammary to left anterior descending coronary artery bypass grafting. Myocardial ischaemia could easily be provoked by selective exercise of the left upper limb. Coronary angiography showed reversal of flow in the left internal mammary artery. Suggestions are given for prevention of the coronary-subclavian steal syndrome by identification of patients who are at risk of developing subclavian artery occlusive disease. Performance of coronary and brachiocephalic angiography is indicated in recurrence of angina in patients with internal mammary artery bypass grafts. Doppler spectral analysis may be a valuable technique for detection of a haemodynamically significant stenosis of the left subclavian artery. Carotid-subclavian bypass grafting is the procedure of choice for management of the coronary-subclavian steal syndrome.

Pulmonary venous obstruction following correction for total anomalous pulmonary venous drainage: a challenge.

Pulmonary venous obstruction after surgical correction of total anomalous pulmonary venous drainage (TAPVD) is a serious condition. Pulmonary venous obstruction can be the result of a primary developmental error or is due to post-operative anastomotic stricture and is usually manifest within 6 months of surgery. Prompt restudy is indicated and if a stricture is present, urgent surgical relief is indicated. However, the results are often disappointing with a high early mortality and a significant chance of restenosis.

Late re-interventions following arterial switch operations in transposition of the great arteries. Incidence and surgical treatment of postoperative pulmonary stenosis.

Seventy-six patients were studied after arterial switch operation (ASO) between May 1977 and February 1992. Pulmonary artery reconstruction was initially performed by: conduit interposition in 5 patients, direct main pulmonary artery anastomosis and button patches in 60 patients, and pantaloon-like patch repair in 11 patients. Pulmonary stenosis developed in 17 patients (22%), requiring a total of 26 late re-interventions. Re-intervention was required in four out of five patients operated with pulmonary artery conduits, 11 out of 60 with a button patch repair and 2 out of 11 following pantaloon-type repair. In this series pulmonary artery stenosis (PS) involving the pulmonary valve occurred in 9/17 patients. Involvement of the pulmonary valve was related to the technique of pulmonary artery reconstruction. In these patients surgery is necessary. Balloon angioplasty can be a valuable tool when the stenosis is more distal. The incidence of PS was not influenced by the type of reconstruction or the use of Lecompte's maneuver.

Perioperative morbidity and mortality of coronary artery surgery after the age of 70 years.

The aim of this study was to compare the actual perioperative (operative and hospital) morbidity and mortality of coronary bypass surgery in patients aged 70 years or older and patients aged 69 years of younger. From January 1987 to September 1988, 102 patients aged 70 years or older underwent isolated aortocoronary bypass surgery. They were compared with the younger patients operated in the same period. Preoperative, operative and postoperative data were analyzed. There was no significant difference between the mortality and morbidity of the two groups. Morbidity was evaluated according to several parameters, low cardiac output, prolonged ventilation, cerebrovascular accident, transient ischemic attacks, pulmonary problems, psychosis, renal failure, wound problems and reoperations. Because this is a limited experience it may be premature to conclude than older patients can be operated on as safely as their young counterparts, but this study shows that age is not in itself a risk factor.

The effectiveness of ketanserin in patients with primary Raynaud's phenomenon. A randomized, double blind, placebo controlled study.

In 41 patients with Primary Raynaud's Phenomenon (PRP) the effectiveness of the serotonin receptor blocker ketanserin has been studied in a double blind cross-over study. Subjective assessments included: frequency and duration of the attacks (both per se and combined to a severity score), cold sensation, numbness, paresthesia, pain, cold water and cold weather provocation and the appearance of spontaneous attacks. The objective measurements comprised Digital Skin Temperature (DST), Digital systolic Blood Pressure (DBP) and Doppler Spectral Analysis (DOSA) of the radial and ulnar arteries. All measurements were performed both at room temperature and after instant cold provocation. The severity score, the occurrence of numbness and paresthesia and cold weather provocation improved significantly on ketanserin treatment. All objective measurements with the exception of the end-diastolic blood flow velocity of DOSA did not show significant improvements. Neither blood chemistry nor systemic blood pressure showed any significant change during ketanserin treatment. However, in the 6 (15%) patients with hypertension both systolic and diastolic blood pressure normalized. Although in objective measurements hardly any significant effects of ketanserin could be demonstrated, the results of the study suggest that orally administered ketanserin is effective for minimizing subjective complaints in patients with PRP. Ketanserin did not show any side effects.

Digital blood pressure and flow measurements in patients with primary Raynaud's phenomenon.

The value of digital systolic blood pressure (DBP) and flow (DBF) measurements for a quantitative diagnosis of Primary Raynaud's Phenomenon (PRP) was evaluated by comparing the results obtained in 10 patients and 20 normals. Digital skin temperature (DST), DBP and brachial systolic blood pressure (BBP) and DBF were measured at room temperature, at maximal vasodilatation and during cold provocation. At room temperature DST discriminates between normals and patients with an accuracy of 77%. On maximum vasodilatation no significant differences can be found in DBP and DBF between normals and patients. However, DBF was significantly different between male and females both in normals and in patients. During gradual cooling a decrease in DBF (a closing phenomenon) was observed both in normals and patients. In contrast DBP did not show a substantial pressure drop. Calculating digit to brachial systolic pressure indexes (DBI) a slight significant increase can be found during cold provocation both in normals and in patients. Only the DBI of digit IV during cold provocation showed a significant difference between normals and patients which resulted in an accuracy of 83%.

Noninvasive hemodynamic assessment of vasospasm in patients with primary Raynaud's phenomenon.

In order to assess vasospastic ischemic disease objectively 41 patients with primary Raynaud's phenomenon and 21 normal volunteers were investigated noninvasively by digital systolic blood pressure and digital skin temperature measurements before and after instant cold provocation. Much care had been taken to be certain that all patients had primary Raynaud's phenomenon. The results of digital pressure measurements appeared to be affected by interindividual variations in systemic systolic blood pressure. By introducing a digit-to-brachial systolic blood pressure index (DBI), such variations could be eliminated. Instant cold provocation did not change DBI significantly. No pressure drop due to a closing phenomenon could be observed. Both digital skin temperature and DBI differed significantly between healthy males and females. In the patient group males and females did not show significant differences. The results in healthy females were hardly different from the results in patients. Healthy males could be well discriminated from patients with both techniques. Sex differentiation appeared to be essential for the objective assessment of primary Raynaud's phenomenon.

En bloc resection for bronchogenic carcinoma with chest wall invasion.

The incidence rate of chest wall invasion in operated bronchogenic carcinoma is about 3%. From 1973 to 1984, 12 patients in our hospital underwent en bloc lung and partial chest wall resection for bronchogenic carcinoma with local invasion of the thoracic wall. All were male, between 49 and 74 years of age. Chest wall or back pain was the most prominent complaint. The duration of symptoms varied from 2 to 20 months. In 10 the tumour was peripherally and in 2 centrally located. Mediastinoscopies, selectively performed were negative. In 11 patients there was a squamous cell and in 1 an adenocarcinoma. 10 lobectomies and 2 pneumonectomies were performed. Macroscopic size of the tumour ranged from 3 to 17 cm, the number of partially resected ribs ranged from 1 to 4. There was 1 operative death (8%). 3 patients died within 5 months and 3 other patients within 14 months. 5 patients are still alive, 2 more than 5 years (17%). The survival is unfavourably influenced by lymph node involvement. The majority of patients became free of pain one month after surgery.

Primary cardiac tumors.

From 1970 to 1986, 3 males and 6 females, ranging in age from 13 to 69 years (median 45 years), underwent heart surgery for a primary cardiac tumor. Six patients had a left atrial myxoma; a lipofibroma, a lymphosarcoma and a rhabdomyosarcoma were found each in 1 patient. The following complaints were present: congestive heart failure in 8, fatigue in 7, cardiac arrhythmia in 3, palpitations in 3, fever in 2 and finally weight loss, nocturnal perspiration and clubbing were each in one patient. The duration of symptoms ranged from 6 weeks to more than 2 years (median 10.8 months). All patients were operated with the aid of extracorporeal circulation as soon as they were diagnosed. One patient with extensive tumor growth died at the end of the procedure. During the follow-up period all patients with a primary malignant tumor died within 3 months. Those with myxoma or fibroma are still alive with a follow-up period extending to 13 years (median 7 years). All surviving patients are asymptomatic and well. Malignant cardiac tumors do have a very poor prognosis, they are only amenable for palliative resection and even then prognosis remains poor. When an endocardial biopsy confirms the diagnosis of a cardiac malignancy, the indication for operation is questionable, although there is an absolute indication in case of obstruction. Cardiac myxoma should be resected after diagnosis because the potential embolic complications may be debilitating or lethal. The operative risk is small, and the long term results are excellent.

[2 successful treatments with extracorporeal membrane oxygenation in neonates with severe respiratory problems]. / Twee geslaagde behandelingen met extracorporele membraanoxygenatie bij pasgeborenen met ernstige ademhalingsproblemen.

We report the successful treatment with extracorporeal membrane oxygenation of two Dutch neonates with severe respiratory insufficiency, due to meconium aspiration syndrome and persistent fetal circulation respectively. During this procedure part of the cardiac output is led outside the body via a venous cannula in the right atrium, oxygenated in a membrane oxygenator, rewarmed to the patient's body temperature in a heat exchanger and returned to the patient via a cannula in the carotid artery debouching into the aortic arch.

[Aortopulmonary window. The need for early surgical correction]. / Aortopulmonaal venster. Noodzaak tot vroege operatieve correctie.

The diagnosis of aortopulmonary window may be problematical. In 7 patients who had undergone operative closure of an aortopulmonary window the diagnostic and operative techniques, the operative findings, and the postoperative course were retrospectively determined. It appeared that the diagnosis is difficult and that cardiac catheterization with angiocardiography is the most accurate diagnostic technique. Good results of operative correction depend on the timing of the operation (preferably before the first year of life), the operative technique, and the severity of additional anomalies of the heart and great vessels. Delay of surgical correction leads to pulmonary hypertension, caused by irreversible pulmonary vascular disease.

[Purulent pericarditis in children. A continuously life-threatening disease]. / Purulente pericarditis bij kinderen. Een nog altijd levensbedreigend ziektebeeld.

Acute purulent pericarditis in children is usually fatal, if not recognized and adequately treated. The history of three children with acute purulent pericarditis is presented. In every septic child who presents with signs of right heart decompensation, acute purulent pericarditis should be seriously considered. Echocardiography is essential for the diagnosis. If possible, diagnostic pericardiocenthesis should be performed. Experience of the last years suggests that excellent results can be obtained when adequate surgical drainage and antibiotic therapy are combined.