You can love it so much. Heart shape.

Diagnostic imaging can sometimes reveal interesting shapes. In this case we describe a transoesophageal echocardiogram of a mitral valve prolapse in shape of a heart sign, which was successfully repaired.

Influence of atrial fibrillation on outcome following mitral valve repair.

BACKGROUND: To investigate the outcome of patients in atrial fibrillation (AF) following mitral valve repair, clinical and echocardiographic follow-up was undertaken in 400 consecutive patients who underwent mitral valvuloplasty from 1987 to 1999. METHODS AND RESULTS: The main indications for surgery were degenerative (81.4%), endocarditis (7.1%), rheumatic (6.6%), ischemic (4.6%), and traumatic (0.3%) mitral valve disease. After excluding 6 paced patients and 1 patient in nodal rhythm, we compared the outcomes of 152 patients in AF against 241 patients in sinus rhythm. For patients in AF versus those in sinus rhythm, more AF patients were older (mean age 67.2+/-8.8 versus 61.9+/-11.8 years, respectively; P<0.001), more were assigned to a poorer New York Heart Association (NYHA) class (77.6% versus 66.0% in NYHA III/IV, respectively; P=0.01), and more demonstrated impaired ventricular function (78.9% versus 46.2% with moderate or severe impairment, respectively; P<0.001). For patients in AF versus those in sinus rhythm, there was no difference in 30-day mortality (2.0% versus 2.1%, respectively; P=0.95), repair failure (5.4% versus 3.6%, respectively; P=0.41), stroke (5.4% versus 2.2%, respectively; P=0.11), or endocarditis (2.3% versus 0.9%, respectively; P=0.27) on follow-up at a median of 2.8 years (interquartile range 1.1 to 6.0). On echocardiography, the proportion of patients with mild regurgitation or worse was 13.3% (AF patients) versus 10.8% (patients in sinus rhythm) (P=0.70). Patients in AF versus those in sinus rhythm had lower survival at 3 years (83% versus 93%, respectively) and 5 years (73% versus 88%, respectively). Univariate analysis identified factors affecting survival as AF (P=0.002), age >70 years (P=0.041), and poor ventricular function (P<0.001). However, by use of a multivariate model, only poor ventricular function remained significant (P=0.01). CONCLUSIONS: AF does not affect early outcome or durability of mitral repair. The onset of AF may be indicative of disease progression because of its association with poor left ventricular function.

Vocal fold palsy due to plombage for tuberculosis.

A 67-year-old patient, who had previously undergone Lucite ball plombage for pulmonary tuberculosis, presented with a hoarse voice, intermittent stridor and breathlessness. Direct laryngoscopy confirmed a left vocal fold palsy. A left supraclavicular mass became apparent and a computerized tomograph (CT) scan showed that a Lucite ball had migrated into her supraclavicular fossa. Subsequently she developed left arm pain and weakness. The balls were removed surgically, following which her arm symptoms improved but her voice remained unchanged. Migration of implanted material should be considered when new symptoms appear in patients who have undergone plombage treatment.

Risk factors for survival following combined heart-lung transplantation. The first 100 patients.

As the numbers of heart and lung transplant recipients have increased it has become possible to identify major risk factors for early (within 3 months) and later (after 3 months) death after this procedure. For 100 patients receiving organs between April 1984 and February 1991, and followed up until February 1992, patient characteristics, operative details, and early morbidity were assessed for their effects on early and later deaths. Recipient age, sex, and preoperative diagnosis did not have a significant effect on early (within 3 months) or later death. Positive cytomegalovirus antibody status of donor or recipient conferred greater risk of death within 90 days (odds ratio [OR] = 3.24, P = 0.06). Greater than 2 L blood in the first 24 hr after operation (OR = 6.00, P = 0.05), and ventilation for greater than 24 hr (OR = 4.87, P = 0.006) were significant prognostic indicators of early death. After the first 3 months, the main risk factor for death was rejection in the first 3 months (OR = 1.38 per episode, P = 0.008). Early infection in general and CMV infection in particular were associated with a small increase in risk. This study confirms the importance of matching donor and recipient for CMV and shows that difficulties during operation, reflected in postoperative bleeding and ventilation times increased the chance of early death. Later death was associated with early acute rejection. A detrimental effect of infection, including CMV infection, either does not exist, or is too small to be detected in a study of this size.

Clinical experience and physiologic results with an implantable intratracheal oxygen catheter.

Ten patients with chronic lung disease received an implanted ITOC. Seven patients continue to use their catheters after a mean period of 14.75 months. Four catheters were removed, 2 at 1 month, 1 after 10 months and 1 after 13 months. One patient requested a second catheter. Three patients experienced mucus plug formation; this was transient in two patients, but led to removal of the catheter in the third. To determine the degree of oxygen-saving afforded by the ITOC, SaO2 was measured at rest and during exercise for eight of the ten subjects using a double-blind technique. The calculated oxygen savings were around 40 percent both at rest and during exercise. The ITOCs were well received by the majority of our patients and were shown to produce a useful saving of oxygen which is of benefit to patients using portable systems and those who require high oxygen flow rates.

Duration of circulatory arrest does influence the psychological development of children after cardiac operation in early life.

Published works on intelligence quotient (IQ) and development following the use of profound hypothermia and circulatory arrest (TCA) to repair congenital heart defects in infants and young children suggest that little or no psychomotor impairment results. IQ scores derived from cognitive, memory, perceptual, quantitative, and verbal tests (McCarthy scale of the children's abilities, mean score 100, SD 16) were measured in 31 patients 5 years following operations performed with TCA between 1972 and 1976. These patients were compared with three control groups: (1) 19 patients with similar defects but operated upon using moderate hypothermia and continuous cardiopulmonary bypass (CPB); (2) 16 children who were the siblings of the TCA patients; and (3) 14 children who were the siblings of the CPB patients. The hypothermic temperatures reached were closely clustered around 15 degrees C in the TCA group and 28 degrees C in the CPB group. TCA time ranged from 22 to 71 minutes. Statistical analysis, which included, t test, chi square test of association, and Wilcoxon test, showed that the only baseline characteristic which differed between the two patient groups in respect to age at operation, age at testing, and preoperative physiological variables (level of cyanosis, weight, oxygen saturation, and hemoglobin concentration) was weight (p = 0.03). The mean score of the TCA group (91 +/- 4.0, SE) was significantly lower (p = 0.002) than that of their siblings (106 +/- 4.1, SE). The score for the CPB patients (102 +/- 5.2, SE) was not demonstrably different from that of their siblings (96 +/- 5.9, SE). The sibling and patient (TCA) IQ differences were associated with duration of arrest in verbal (p = 0.06), quantitative (p = 0.07), and general cognitive (p = 0.003) scores. A decrease of 0.53 point per minute of arrest time was estimated for the entire group of 31 patients; that is, in the 19 patients with siblings, for each minute increase in circulatory arrest time, the patients dropped 0.69 IQ point below their siblings. These results and analysis of other published data do not support the generally accepted view that TCA can be used entirely without penalty. We question the accepted "safe" limit of circulatory arrest of 60 minutes.

Hemodynamic evaluation of the Monostrut and spherical disc Björk-Shiley aortic valve prosthesis with Doppler echocardiography.

A Doppler echocardiographic study was performed to assess whether the Monostrut model of the Björk-Shiley valve (Shiley, Inc., Irvine, Calif.) had an improved hemodynamic performance in comparison with the spherical disc model in the aortic position. Twenty retrospectively randomly selected patients were studied, 10 with each valve type. Within each valve type two sizes of valve were studied, 21 and 23 mm. The two groups were comparable with respect to age, postoperative time, fractional shortening, New York Heart Association functional class preoperatively, and body surface area. Pulsed and continuous wave Doppler measurements were recorded at rest. Continuous wave Doppler recordings were performed every 2 minutes after exercise with supine bicycle ergometry until 10 minutes after exercise. Peak and mean gradients across the aortic valve prostheses were estimated. Both groups achieved a significant and comparable rise in heart rate with exercise. The mean gradients +/- standard error of the mean at rest and 2 minutes after exercise were 19.7 +/- 1.9 mm Hg and 30.9 +/- 2.2 mm Hg, respectively in the spherical disc group compared with 14.9 +/- 1.1 mm Hg and 23.6 +/- 1.7 mm Hg in the Monostrut group (p < 0.05 and p < 0.025, respectively). Peak transvalvular gradient at rest was 30.7 +/- 2.7 mm Hg in the spherical group compared with 23.9 +/- 1.9 mm Hg in the Monostrut group (p < 0.05). We conclude that the Monostrut Björk-Shiley valve prosthesis has better hemodynamic performance than the spherical disc model in the aortic position.

Selection and procurement of combined heart and lung grafts for transplantation.

Between April 1984 and July 1986, 14 patients underwent heart-lung transplantation at Papworth Hospital, Cambridge, England. The donors for the first five operations were brought to our hospital and the organs removed in the operating theater adjacent to that in which the recipients were prepared. Subsequently, organs have been procured from distant centers. The total ischemic time ranged from 48 to 51 minutes (mean 49.6) for the near procurement group and from 70 to 186 minutes (mean 123.6) for the distant procurement group. Our method of preservation consists of cold cardioplegic arrest of the heart with St. Thomas' Hospital solution followed by a single cold (4 degrees C) pulmonary artery flush with a solution containing 500 ml donors blood, 700 ml Ringer's solution, 200 ml 20% salt-poor albumin, 100 ml 20% mannitol, 20 micrograms prostacyclin, and 10,000 units heparin. Function of the lungs after implantation was assessed by measuring the alveolar-arterial oxygen gradient. The median alveolar-arterial oxygen gradient measured shortly after discontinuation of bypass (point 1), just before extubation (point 2), and at 1 week (point 3) were 96.0, 62.3, and 18.8 mm Hg, respectively, for the near procurement group and 91.5, 60.0, and 11.3 mm Hg, respectively, for the distant procurement group. Comparison of the two groups at the three measurement points by the nonparametric Wilcoxon test showed no significant difference (p = 0.44, 0.52, and 0.11, respectively). The two groups showed significant decline of the alveolar arterial oxygen gradient differences over the first week (p = 0.004, nonparametric Friedman test). We conclude that our method of preservation provides a satisfactory function after implantation. The alveolar-arterial oxygen gradient differences were high immediately after implantation but decreased significantly afterward.

Audit of referral and explant diagnoses in lung transplantation: a pathologic study of lungs removed for parenchymal disease.

BACKGROUND: Lung transplantation is performed for an increasing range of pulmonary conditions in which the diagnosis is often clinical or based on limited biopsy material. Diagnosis may be made late in the course of the disease where specific features are no longer present. Posttransplantation complications and disease recurrence may relate to the primary disease, and accurate diagnosis is therefore essential. METHODS AND RESULTS: A pathologic review of 183 explanted lungs over a 10-year period (heart-lung = 109, single lung = 65, double lung = 9) showed 29 significant discrepancies or additional features likely to effect outcome. The final pathologic diagnosis was cystic fibrosis (n = 66), emphysema (59), bronchiectasis (17), pulmonary fibrosis (19), sarcoidosis (10), Langerhans cell histiocytosis (3), pulmonary veno-occlusive disease (3), posttransplantation obliterative bronchiolitis (2), primary hemosiderosis (1), rheumatoid obliterative bronchiolitis (1), extrinsic allergic alveolitis (1), pneumoconiosis (1). Unsuspected diagnoses included tuberculosis (8) (four cases of which were active and in single lung recipients requiring antituberculous chemotherapy), sarcoidosis (9), (of which, six were unsuspected primary diagnoses and three were additional diagnoses), veno-occlusive disease (3), carcinoma (1), pneumoconiosis (1), and pulmonary fibrosis (2). Aspergillus infection (2) and bronchocentric granulomatosis (3) were found in patients with cystic fibrosis. One active tuberculosis case also showed an aspergilloma. Unsuspected infections requiring therapy in immunosuppressed patients and previously unsuspected sarcoidosis, which is known to recur in the graft, were the major novel diagnoses. Discrepancy rate was 12 of 65 in single lungs (19%) and 17 of 109 in heart-lungs (16%). CONCLUSIONS: These results emphasize the need for accurate preoperative diagnosis especially when the similarly diseased native lung remains in situ.

Domino heart transplantation: the Papworth experience.

The use of donor hearts from heart-lung recipients, the so-called domino procedure, began at Papworth Hospital in November 1988. Between then and September 1992, 198 heart transplantations and 86 heart-lung transplantations were performed. Fifty-three heart-lung recipients donated their hearts for use in the domino procedure. Thirty-two domino hearts were transplanted at Papworth and 21 were exported to other centers. Institution of the domino procedure allowed us to perform 19% more heart transplantations (166 to 198) than would have been done had the procedure not been used. The ischemic time was significantly shorter for the domino hearts compared with organs from brain dead donors (134 minutes versus 191 minutes; p < 0.001). No difference was found in the 3-month (84% versus 83%) or 1-year (74% versus 76%) survival between domino and nondomino recipients. Other potential advantages of the domino procedure include detailed pretransplantation evaluation of the heart in live donors and the potential for human leukocyte antigen matching. Additionally many heart-lung recipients have elevated pulmonary artery pressures and a "conditioned", hypertrophied right ventricle. The use of such hearts for heart transplantation has theoretic appeal for patients with elevated pulmonary vascular resistance.

Heart-lung transplantation for end-stage respiratory disease in patients with cystic fibrosis at Papworth Hospital.

Between October 1985 and July 1992 we performed heart-lung transplantation in 42 patients with end-stage respiratory disease caused by cystic fibrosis. Twenty-eight of these patients are alive at 3 months to 7 years after heart-lung transplantation. Actuarial survival at 1 year and 3 years after transplantation was 78% and 65%, respectively, in this group, which compares favorably with 77% and 60%, respectively, in patients without cystic fibrosis undergoing heart-lung transplantation. Similarly no significant difference was found in the incidence of postoperative infections between patients with cystic fibrosis and patients without cystic fibrosis undergoing heart-lung transplantation. Quality of life indexes analyzed by the Nottingham Health Profile reveal significant improvements for patients surviving longer than 3 months from surgery. Long-term survival is determined by the development of obliterative bronchiolitis, which has occurred in 11 patients, six of whom have died. Early postoperative survival appears to be compromised by poor preoperative nutritional status. The study provides evidence that patients with end-stage respiratory disease caused by cystic fibrosis gain a survival advantage and improvement in quality of life after heart-lung transplantation when compared with patients who do not receive this form of therapy.

Airway stenoses after lung transplantation: management with expanding metal stents.

Success in lung transplantation has been hindered by airway complications, usually as a result of anastomotic ischemia and stenosis. We report our experience with expanding metal stents in managing airway stenoses after lung transplantation. From April 1984 through November 1993, 46 single lung, 5 double lung, and 154 heart-lung transplantations were performed at Papworth Hospital. All patients received immunosuppression with azathioprine, cyclosporine, methylprednisolone, and induction antithymocyte globulin. Fourteen patients (nine single lung, two double lung, and three heart-lung) had an airway stenosis requiring a stent. The most common features were shortness of breath, wheezing or stridor, and a fall in pulmonary function tests (11 patients). Three patients had pneumonia. Airway stenosis was diagnosed on bronchoscopy an average of 61 days after transplantation (range 3 to 245 days). Stent placement occurred an average of 18 days after the diagnosis (range 2 to 84 days). One heart-lung transplant recipient received a silicone rubber stent. All other patients received expanding metal stents. Six patients required multiple stent placements. After stent placement the average increase in the forced expiratory volume in 1 second was 117%. Infection complicated the stenoses in 12 patients. Pseudomonas aeruginosa and Aspergillus fumigatus were the most common pathogens, each occurring in six cases. Multiple pathogens were isolated in seven cases. Three patients died as a direct consequence of their airway problems. Two died of pneumonia despite stenting, and a third died of acute occlusion of the silicone rubber stent. Expanding metal stents are an effective treatment of airway stenoses in lung transplant recipients. Patients with suspected airway problems should be referred for early bronchoscopy with the potential for stent placement.

Lung Transplantation in patients with systemic diseases: an eleven-year experience at Papworth Hospital.

BACKGROUND: The presence of a systemic disease has traditionally been considered a contraindication to lung transplantation. METHODS: We present a retrospective review of 19 patients undergoing lung transplantation for end-stage pulmonary disease associated with a systemic illness since 1984. There were 11 male and 8 female patients, aged from 23 to 59 years (median 43 years) with end-stage pulmonary involvement by sarcoidosis (11 patients), Langerhan's cell histiocytosis (three patients), systemic vasculitis (four patients: three with systemic lupus erythrematosis, one with Churg-Strauss), and common variable immunodeficiency (one patient). Ten patients received a heart-lung transplant, and eight patients received a single lung transplant. One patient underwent single lung transplantation after an earlier heart-lung transplant. RESULTS: The 30-day mortality was 5.3%. Nine patients died overall. Two of these had systemic lupus erythrematosis with anticardiolipin antibodies and died from complications of their underlying vasculitis. The mean 1- and 2-year actuarial survivals for all patients were 71% (standard error +/- 10.8%) and 64% (standard error +/- 11.9%), respectively. All patients surviving longer than 3 months achieved an improvement in functional status to New York Heart Association class I or II, and a significant increase occurred in mean forced expiratory volume in 1 second and forced vital capacity. Disease recurrence without clinical significance occurred in two patients with sarcoidosis. Of the nine patients who died, seven had autopsies and none showed evidence of disease recurrence in the lungs. CONCLUSIONS: Patients with systemic diseases can be considered for lung transplantation and each case should be judged on its individual merits. However, patients with systemic lupus erythrematosis (particularly when associated with anticardiolipin antibodies) should probably not be offered lung transplantation because they are likely to develop further complications of their underlying vasculitis.

Use of prostacyclin and ultrafiltration in adult respiratory distress syndrome.

A primigravida with severe kyphoscoliosis developed cardio-respiratory failure in pregnancy. Cardiac arrest occurred 10 days after Caesarean section; gastric acid was aspirated then and was followed by the development of adult respiratory distress syndrome. Initial recovery, with clearing of peripheral oedema, was followed by a recurrence of respiratory distress associated with infection. Profound hypoxaemia and oliguria unresponsive to diuretics were relieved by the infusion of prostacyclin combined with fluid removal by ultrafiltration. This treatment may be of value in the management of respiratory distress syndrome when pulmonary oedema is the dominant feature.

Traumatic intercostal pulmonary hernia.

Traumatic intercostal pulmonary hernia is a rare sequel to injury, especially in children and where there is no penetrating wound. Spontaneous regression of these hernias has been reported and conservative management has been advocated. We describe a case in which a smooth-walled intercostal defect and lung hernia that did not regress developed in a child. This was successfully repaired with a Gore-Tex (expanded polytetrafluoroethylene) patch. Repair is advisable in such cases, but operation can be delayed to allow possible resolution.

Conservative surgical treatment of valvular injury after blunt chest trauma.

BACKGROUND: Blunt injury to the cardiac valves leads to progressive ventricular failure often requiring surgical management. Most frequently, prosthetic replacement is the chosen management. METHODS: Three consecutive patients presenting to one surgeon with blunt traumatic valve lesions formed the study group. RESULTS: At operation, the valvular pathology was assessed, and reparative techniques were used to correct the defects. All the patients had an excellent outcome at follow-up periods of 2 to 3 years. CONCLUSIONS: Conservative operation to repair traumatic valve lesions is feasible and has potential advantages over replacement.

A multidisciplinary approach to primary nonseminomatous germ cell tumors of the mediastinum.

The introduction of cis-platinum-based chemotherapy has dramatically improved the prognosis for patients with primary nonseminomatous germ cell tumors of the mediastinum. Since 1978, 12 male patients (mean age, 29 years) have been seen with a large mediastinal mass, normal testes, and abnormal testicular tumor markers. Eleven patients had raised alpha-fetoprotein levels (median, 1,300 micrograms/L; normal, less than 10 micrograms/L), and 3 had elevated levels of the beta fraction of human chorionic gonadotropin (median, 8,000 IU/L; normal, less than 5 IU/L). Two patients were treated by primary surgical intervention followed by chemotherapy. Ten patients were treated with primary chemotherapy (cis-platinum, vinblastine sulfate or etoposide, and bleomycin sulfate), and this was followed by timed surgical excision of the tumor mass in 7. Six (60%) patients responded to primary chemotherapy with normalization of tumor markers. In this group there was 1 postoperative death and 1 recurrence. The 4 remaining patients are alive and free from disease at a mean of five years. Of the 4 patients with persistently elevated tumor markers, 2 died within six months, 1 is alive with recurrence, and 1 is lost to follow-up at three months. Patients whose tumor markers return to normal after cis-platinum-based chemotherapy have a good long-term prognosis following radical surgery. If the tumor markers remain elevated, the prognosis is poor.

Surgical correction of postpneumonectomy stridor by saline breast implantation.

Postpneumonectomy syndrome is a rare complication of pneumonectomy and is characterized by progressive dyspnea, stridor, and repeated chest infections. It is caused by displacement and rotation of the mediastinal structures into the pneumonectomy space, producing compression and malacic changes in the trachea and remaining bronchus. We report the successful long-term results of mediastinal correction, cardiopexy and plombage with saline breast prostheses in a 59-year-old man after right pneumonectomy for carcinoma of the lung.