Håkan Berggren

Total Anomalous Pulmonary Venous Connection: Morphology and Outcome From an International Population-Based Study

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. # Clinical Perspective {#article-title-32}

Centralization of Pediatric Heart Surgery in Sweden

Abstract. In Sweden, which has a population of 8.9 million people, pediatric heart surgery was previously performed in four cities. After a long, difficult process, centralization of pediatric heart surgery to two centers was achieved in 1993. The overall 30-day mortality for open-heart surgery on infants and children of 9.5% before the centralization (1988–1991) was reduced to 1.9% in 1995–1997. A causal relationship between the mortality rates before and after the centralization is impossible to prove. Heart surgery was concentrated to the two centers with the lowest surgical mortality, and the reduction in surgical mortality was observed over a short period of time which makes it likely that the centralization of the surgical activity promoted the improved results. During the later time period the amount of more complex surgery was clearly increased compared to that performed previously.

Hemofiltration modifies complement activation after extracorporeal circulation in infants

Complement and fibrinolytic factors were measured in 9 infants undergoing hemofiltration immediately after cardiopulmonary bypass in an attempt to reduce activation of these systems. Plasma levels of C3a, C5a, and terminal complement complexes increased during bypass by 460%, 85%, and 745%. Plasma levels were reduced after hemofiltration in 8 of the 9 infants, and C3a and C5a fractions were recovered in the ultrafiltrate. The observed activation of the fibrinolytic system seemed to be unaltered by hemofiltration. Fibrinolytic factors were not filtered. Our study shows that increased concentrations of complement factors in the plasma after bypass in infants may be reduced by hemofiltration.

Intraoperative thromboelastometry is associated with reduced transfusion prevalence in pediatric cardiac surgery.

BACKGROUND:The majority of pediatric cardiac surgery patients receive blood transfusions. We hypothesized that the routine use of intraoperative thromboelastometry to guide transfusion decisions would reduce the overall proportion of patients receiving transfusions in pediatric cardiac surgery. METHODS:One hundred pediatric cardiac surgery patients were included in the study. Fifty patients (study group) were prospectively included and compared with 50 procedure- and age-matched control patients (control group). In the study group, thromboelastometry, performed during cardiopulmonary bypass, guided intraoperative transfusions. Intraoperative and postoperative transfusions of packed red blood cells, fresh frozen plasma, platelets, and fibrinogen concentrates, and postoperative blood loss and hemoglobin levels were compared between the 2 groups. RESULTS:The proportion of patients receiving any intraoperative or postoperative transfusion of packed red blood cells, fresh frozen plasma, platelets, or fibrinogen concentrates was significantly lower in the study group than in the control group (32 of 50 [64%] vs 46 of 50 [92%], respectively; P < 0.001). Significantly fewer patients in the study group received transfusions of packed red blood cells (58% vs 78%, P = 0.032) and plasma (14% vs 78%, P < 0.001), whereas more patients in the study group received transfusions of platelets (38% vs 12%, P = 0.002) and fibrinogen concentrates (16% vs 2%, P = 0.015). Neither postoperative blood loss nor postoperative hemoglobin levels differed significantly between the study group and the control group. CONCLUSIONS:The results suggest that routine use of intraoperative thromboelastometry in pediatric cardiac surgery to guide transfusions is associated with a reduced proportion of patients receiving transfusions and an altered transfusion pattern.

Hospital Mortality and Long-term Survival in Relation to Preoperative Function in Elderly Patients with Bronchogenic Carcinoma

During an eight-year period, 82 patients 70 years of age or older were operated on for bronchogenic carcinoma. Hospital mortality was 15.9%, and five-year survival was 32%. Results of preoperative dynamic spirometry and bicycle ergometry were predictive for post-operative six-week mortality but not for long-term survival.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. # Clinical Perspective {#article-title-51}Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ⩽18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

Influence of two different perfusion systems on inflammatory response in pediatric heart surgery

BACKGROUND This study tests the hypothesis that a cardiopulmonary bypass system that combines complete heparin-coating, a centrifugal pump, and a closed circuit in comparison with a conventional system (uncoated system, roller pump, and hard shell venous reservoir) attenuates the inflammatory response in pediatric heart surgery. METHODS In a prospective randomized controlled clinical study 40 consecutive children weighing 10 kg or less were included and divided into two groups. Concentrations of complement proteins (C3a, sC5b-9, C4d, and Bb), granulocyte degranulation products (polymorphonuclear [PMN] elastase), and proinflammatory cytokines (tumor necrosis factor [TNF]-alpha, interleukin [IL]-6, and IL-8) were measured. RESULTS C3a and sC5b-9 concentrations were lower (C3a, p < 0.001; sC5b-9, p = 0.01) in the combined (heparin-coated/centrifugal pump/closed reservoir) group, the peak values being 58% and 37% of conventional group values. The Bb- and C4d-fragment values indicated activation of the complement system through the alternative pathway in both groups. PMN elastase concentrations were lower (p = 0.02) in the combined group, the peak values being 43% of conventional group values. There were no significant intergroup differences regarding TNF-alpha, IL-6, or IL-8 concentrations. CONCLUSIONS The use of a fully heparin-coated system, a centrifugal pump, and a closed circuit during CPB in children (10 kg or less) leads to a lower degree of complement activation and PMN elastase release compared with a conventional system.

Thermographic Demonstration of Uneven Myocardial Cooling in Patients with Coronary Lesions

Low temperature is an important factor in protecting the myocardium during an operation on the heart. This can be difficult to accomplish if the cold cardioplegic solution is hindered by occlusions or stenosis of the coronary arteries. We used thermography to study myocardial temperature during infusion of cold cardioplegic solution. Slow cooling was recorded distal to coronary stenosis or occlusions, thereby indicating insufficient protection of the myocarium in these areas.

Long-term follow-up of operative treatment for pulmonary metastases

From 1954 onwards, 132 patients underwent 165 resections for pulmonary metastases. No other forms of therapy were adopted. The operative mortality was 7.6% (10 patients). After a minimum follow-up of 5 years, the overall survival rate was 20%. The 10- and 15-year survival rates were 6% and 3%, respectively. The major cause of late death was recurrence or spread of the primary disease in 102 patients (83.6%) while 5 patients died of reasons not related to cancer. Fifteen patients (12.3%) are still alive, 13 of whom have no clinical cancer. The presence of symptoms, the disease-free interval of less than 1 year and the number of metastases showed a statistically significant detrimental influence on survival (P less than 0.001, P less than 0.05 and P less than 0.046, respectively). The best 5-year survivals of 42%, 24%, 23% and 23% were noted for metastases from malignancies in the body of the uterus, kidney, bone and colon, respectively. Longterm survival or clinical cure can be achieved with surgery alone by an aggressive approach in selected malignancies.

Surgical Removal of Pulmonary Metastases from Renal Cell Carcinoma

Thirty-three patients operated on for pulmonary metastases from renal cancer were followed up for a minimum of 5 years or to death. The 5-year survival was 21%. There was a tendency to better survival in patients operated by lobectomy rather than limited resection. Extended operations carried a grave prognosis. Manifest metastatic disease within one year after the primary operation showed shortened survival. Repeated operations were possible, with good results. It is concluded that operations for pulmonary metastases can be performed with good results. However, the effect is a palliative one as the ultimate cause of death in all instances was the spread of the cancer disease.