Building a pulmonary vascular service: the 12-year experience and outcomes of the Auckland pulmonary arterial hypertension clinic.

BACKGROUND AND AIMS: Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality. In New Zealand, access to PAH-specific pharmacotherapy has been restricted for economic reasons. Since 2009, access to medication has been provided by a Special Authority scheme that ensures equity of access for all patients. Management of patients with PAH in this environment poses unique challenges. This study describes outcomes of patients managed in the Auckland PAH clinic over a 12-year period. METHODS: The clinic database was interrogated. Patients were eligible for this study if they had PAH (World Health Organization (WHO) Class 1, 1' and 4) and had been managed in the clinic from the year 2000. RESULTS: One hundred and twenty-six patients (75% female, mean age at diagnosis 50) were included. Most had idiopathic PAH (37%) or PAH because of connective tissue disease (29%). At diagnosis, patients had advanced disease (median WHO Class III, 6-min walk distance 367 m and pulmonary vascular resistance of 10.6 Wood Units). Initial therapy was sildenafil in most cases. PAH-specific therapy was associated with improved WHO class and longer 6-min walk distance (P < 0.01 for both). Thirty per cent of patients were escalated to combination therapy. Survival was 91% at 1 year and 67% at 5 years. CONCLUSIONS: Despite historic difficulties with access to PAH-specific therapy, these data confirm benefits of therapy (primarily sildenafil as first-line treatment) for patients with PAH managed within the Auckland PAH clinic. Survival data are comparable with other reported cohorts.

Doppler echocardiography in the assessment of the homograft aortic valve.

To determine the utility of Doppler echocardiography in the evaluation of the homograft valve in the aortic position, 27 patients with normally functioning valves (group 1) and 30 patients with suspected malfunctioning valves (group 2) were examined. Simultaneous cardiac catheterization and Doppler echocardiography were performed in 23 group 2 patients. Doppler and surgical findings were compared in 7 patients too ill for invasive studies. In group 1 patients, the maximal velocity (+/- standard deviation) was 1.8 +/- 0.37 m/s, the mean pressure gradient was 7.1 +/- 3.07 mm Hg and the mean aortic valve area was 2.2 +/- 0.79 cm2. The maximal velocity in group 2 patients with aortic regurgitation (AR) classified as moderate or greater was 2.5 +/- 0.55 m/s, compared with 1.8 +/- 0.44 m/s in patients with mild AR or less (p less than 0.01). In the quantitation of AR, pulsed-wave mapping and angiographic grades were identical in 18 patients and differed by 1 grade in 5. Seven patients too ill for catheterization had severe destruction of valve leaflets at cardiac surgery. In 6 patients, both Doppler grading methods suggested severe AR. In a seventh patient, who had an obstructed Starr-Edwards valve in the mitral position, AR was graded as mild by pulsed-wave mapping. Only 1 patient had homograft valve stenosis, with a withdrawal gradient at catheterization of 34 mm Hg and a Doppler maximal gradient of 36 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Rest and exercise hemodynamics of 20 to 23 mm allograft, Medtronic Intact (porcine), and St. Jude Medical valves in the aortic position.

Doppler echocardiography was used to measure gradients and valve areas at rest and after supine bicycle exercise in 35 patients with valve replacements 20 to 23 mm in size. Thirteen patients with a St. Jude Medical valve (St. Jude Medical, Inc., St. Paul, Minn.) were matched to 13 patients with an allograft valve, and seven patients with a Medtronic Intact (porcine) valve (Medtronic, Inc., Minneapolis, Minn.) to seven patients with an allograft valve. Patients were matched for age, sex, valve size, body surface area, and left ventricular systolic function. There was no statistically significant difference between the matched groups for body surface areas, resting cardiac output, exercise heart rate, or workload achieved. Mean pressure gradient was higher for St. Jude Medical than for allograft groups, both at rest (11.8 +/- 6.67 mm Hg for St. Jude Medical versus 6.67 +/- 2.98 mm Hg for allografts) and after exercise (16.4 +/- 8.47 mm Hg versus 9.7 +/- 3.94 mm Hg), but the differences were of borderline significance (p = 0.016 and 0.027, respectively). Valve area at rest was similar for both devices (1.4 +/- 0.45 cm2 for St. Jude Medical versus 1.8 +/- 0.56 cm2 for allograft; p greater than 0.1). There were highly significant differences between patients with Intact and those with allograft valves for resting mean pressure gradient (19.3 +/- 4.23 mm Hg for Intact versus 5.9 +/- 3.68 mm Hg for allograft; p less than 0.001) and for exercise mean pressure gradient (27.8 +/- 8.63 mm Hg versus 8.1 +/- 8.43 mm Hg; p less than 0.001). The differences between the valve areas at rest also were significant (1.1 +/- 0.12 cm2 versus 2.2 +/- 0.62 cm2; p less than 0.01). It is concluded that when a tissue valve is indicated in patients with a small aortic root, the freehand allograft aortic valve is an ideal device from the hemodynamic perspective and is superior to the Intact valve. It is also probably superior in this respect to the St. Jude Medical valve, although the analysis may be biased slightly in favor of the allograft valve.

Early follow-up of patients with the Medtronic Intact porcine valve. A new cardiac bioprosthesis.

A new-generation porcine valve fixed in glutaraldehyde at zero pressure and mounted on an acetal copolymer flexible stent was inserted in 97 patients between August 1983 and October 1986. The mean age of the patients was 51 years (range 10 to 76) and eight were under the age of 20 years. There were 57 mitral, 33 aortic, and 10 tricuspid valve replacements. Concomitant coronary artery bypass grafting was performed in 9% of patients, 40% underwent multiple valve operations, and in 40% the procedure was a reoperation. Mean follow-up was 26 months (range 12 to 49) and was 99% complete. There were no examples of primary tissue failure, and only to reoperations have been undertaken for infective endocarditis alone. The early mortality rate was 8.2% and the late mortality rate, 12.1%. Four late deaths were valve related (two caused by infective endocarditis and two by embolism). The actuarial 3-year survival rate was 70%, freedom from infective endocarditis 879%, freedom from embolism 87%, freedom from reoperation 90%, and freedom from valve-related complications 77%. All but three surviving patients were in New York Heart Association class I or II. Doppler echocardiography, performed in 62 of 76 survivors, showed thin and mobile leaflets in all patients and trivial or mild regurgitation in four (6%). The mean gradient across the Medtronic Intact valves (Medtronic Blood Systems Inc., Minneapolis, Minn.) in the aortic position was 17 +/- 5.2 mm Hg, in the mitral position 3.8 +/- 1.33 mm Hg, and in the tricuspid position 4.1 +/- 1.14 mm Hg. We conclude that early results with the Intact valve are encouraging.

Survival after heart transplantation without regular immunosuppression.

The case of a young woman who discontinued regular immunosuppression 4 years after heart transplantation is described. A year later she became unwell and explained her actions. A cardiac biopsy showed changes of focal moderate rejection, which responded to treatment, and she is presently in good health.

Aortic valve replacement in the elderly.

Aortic valve replacement is an established mode of therapy that enhances and prolongs life in patients with significant aortic valve disease. This is true for the elderly, but with a higher reported mortality and morbidity than in younger patients. One hundred and eighty-three elderly patients aged 70 years and over were considered suitable candidates for isolated aortic valve replacement (n = 97) or aortic valve replacement combined with coronary artery bypass surgery (n = 86) in the decade 1980-89. The mean age at surgery was 73.2 years (70-80). The hospital mortality and morbidity of this group were compared to 340 consecutive patients aged 60-69 who underwent similar surgery over the same period. There was no significant difference in hospital mortality: 4.4% for elderly patients and 4.1% for those aged 60-69. Measures of hospital morbidity were similar except for the perioperative stroke rate, which was 4.4% in the elderly patients and 1.5% in the younger group (p < 0.05). Detailed long term follow up was obtained in 75 of the 76 elderly patients who underwent aortic valve replacement or aortic valve replacement combined with coronary artery bypass surgery in the first five year period. Hospital survival was 99% (one hospital death). The overall five year actuarial survival was 81% and greater than the age and sexmatched general population (73%). Of the 44 patients alive at the time of review, 35 (80%) were free of angina and 32 (73%) had no significant symptoms of heart failure. Additionally, these patients were questioned about their quality of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Transoesophageal echocardiography in patients with prosthetic heart valves and systemic emboli: is it a useful investigation?

AIM: We aimed to assess the impact of transoesophageal echocardiography (TOE) on the clinical management of patients with prosthetic heart valves who had suffered from a systemic embolus. We wanted to know whether the TOE examination actually changed the management of these patients. METHODS: Prospective assessment of 38 TOE studies, with retrospective chart review of the hospital treatment. RESULTS: In 16 TOE studies, no potential cardiac source of emboli was found, however in 22 studies a cardiac abnormality was detected. As a result of the TOE, in 13 cases there was a definite change in clinical management for the patient. CONCLUSION: A TOE is an important examination for patients with a prosthetic valve who present with a systemic embolus.

Cardiac transplantation in New Zealand: eight years experience.

Between December 1987 and December 1995, 62 patients underwent orthotopic cardiac transplantation at Green Lane Hospital. Their cardiac dysfunction resulted from dilated cardiomyopathy (32), coronary artery disease (21), rheumatic heart disease (7), congenitally corrected transposition of the great arteries (1) and hypertrophic cardiomyopathy (1). Before transplantation all patients were in New York Heart Association (NYHA) functional class III or IV. Eight patients (13%) died in the perioperative period and a further seven (11%) died 1 to 4 years after transplantation. Actuarial 1 and 3 year survival was 87% and 80% respectively. Forty-five of forty-seven surviving patients were in NYHA functional class I at the time of analysis. The results confirm that cardiac transplantation has a limited but valuable role in the treatment of end stage myocardial dysfunction.

Heart donation: the New Zealand experience, 1987-1996.

AIMS: To audit all donor hearts offered to our unit, assess the outcome, identify factors which might enhance the acceptance rate and provide data on which to base and modify acceptance criteria. METHODS: Demographic, clinical and outcome data were collected retrospectively and analysed for all patients in whom brain death had been established and permission for organ donation obtained from the family prior to referral to the transplant donor coordinator. RESULTS: Over a nine year period (1987-96) 267 hearts were offered for transplantation. The mean donor age was 31.2 years (2-72), 163 being male and 104 female. The cause of death was secondary to trauma in 52%, neurological in 44% and due to hypoxia in 4%. The mean donor weight was 68 kg and the distribution of ABO blood group similar to the New Zealand population. Seventy-six hearts (28%) were implanted. Of the 191 not implanted no suitable recipient was available for 98 (51%) according to blood group and body weight criteria, 38 donors (20%) were medically unsuitable, 37 (19%) were declined because of donor age, 16 (8%) because of a markedly abnormal echocardiogram and 2 (1%) for logistical reasons. CONCLUSIONS: Enhanced efforts are needed to increase public awareness of the benefits of organ donation. However, a greater number of heart transplants may be achieved by increasing the recipient pool.

Outcome following heart transplantation in Maori and Polynesian patients: a comparison with European New Zealanders.

AIM: To compare demographic, clinical and outcome data of Maori and Polynesian with New Zealand European heart transplant patients. METHODS: A retrospective analysis was made of data from the 104 patients who underwent heart transplantation at Green Lane Hospital over a period of twelve years, of whom 79 were European, 23 Maori/Polynesian, and two Asian. Clinical characteristics, blood group, HLA matching and outcomes of recipients were compared. RESULTS: There was no significant difference in age and gender between the two groups. Maori and Polynesian patients were heavier, had a greater body mass index and were more likely to have rheumatic heart disease than their European counterparts. Maori/Polynesian patients were predominantly blood group A, whilst European patients were mainly group O. The waiting time for a donor heart was similar in both groups. There was no significant difference in number of rejection episodes and survival. CONCLUSIONS: Green Lane Hospital has the largest international experience of heart transplantation in Maori and Polynesian patients. Although there are some differences in clinical profile, outcome in terms of rejection episodes and survival is similar in the two groups.

Cardiac transplantation in New Zealand: an initial report.

During the first 18 months of its establishment, the cardiac transplant unit at Green Lane Hospital has undertaken 10 transplant operations. Fifty potential recipients have been referred and 15 were accepted for transplantation. Of the ten who have had surgery, 2 are making good progress at school, 1 is active at home and 5 have returned to work. Two patients have died.

Cardiac allograft vasculopathy: the green lane hospital experience 1987-1998.

AIMS: To determine the prevalence of cardiac allograft vasculopathy in heart transplant recipients at Green Lane Hospital and to examine potential risk factors for vasculopathy. METHODS: We retrospectively reviewed the coronary angiograms of all cardiac transplant recipients. Angiography was usually performed one, two and five years after operation. The diagnosis of allograft vasculopathy was made if there was any evidence of coronary artery disease. Patients' medical records were reviewed for potential risk factors. RESULTS: Ninety-one patients underwent cardiac transplantation between December 1987 and March 1998. One year survival was 87%. Angiographic evidence of coronary disease was present in 30 patients and in three patients coronary lesions were first identified at post mortem. Vasculopathy was present in 25%, 35% and 61% of patients at one two and five years following transplant. Donor-acquired lesions could not be excluded as few patients had immediate postoperative angiograms for comparison. Five late deaths have been due to vasculopathy. Recipient age, race, donor age and ischaemic time were similar for those with and without vasculopathy. Frequency or severity of acute rejection episodes, cytomegalovirus infection, lipid profiles, diabetes and hypertension were not significantly different in patients with vasculopathy. CONCLUSION: Cardiac allograft vasculopathy is a common finding after heart transplantation. No definite risk factors were identified in this patient group.

Return to work after heart transplantation: the New Zealand experience.

INTRODUCTION: Return to work and social re-integration following heart transplantation is a significant challenge for patients. The aim of this study is to provide a snapshot of the current employment status and factors associated with return to work in New Zealand recipients over the past decade. METHODS: Consecutive surviving patients who underwent heart transplantation in the 10 years from June 2001 to June 2011, alive in July 2012, were retrospectively identified. Details on demographics, employment before and after transplantation were obtained and recorded. RESULTS: A total of 87 patients were included, out of a total of 111 patients who underwent heart transplantation in the 10 year period from June 2001 (24 patients had died prior to July 2012). The median age of the study cohort was 52 years (range 15-75 years) and 19 were female. A total of 51 (58.6%) patients were in paid employment at the time of review. Of the 36 (41.4%) patients not in paid employment, 5 were students, 12 were retired and 10 were homemakers or not working through lifestyle choice. Two patients were unable to work for health reasons. Seven (8%) patients considered able to work were on an unemployment or invalid's benefit. Of the patients working prior to heart transplantation, 88.9% returned to work after a median of 8.5 months, and 70.6% remained on paid employment at a median follow-up of 77 months after transplantation. There was a statistically significant correlation between the time of stopping work prior to transplant and return to work after transplant (r = 0.497, P < .01). CONCLUSION: The current rate of paid employment in patients who underwent heart transplantation was similar to the overall employment rate in New Zealand. The most important predictor of returning to work was employment status prior to transplantation. Discussions regarding return to work early in the transplantation assessment process and actively assisting patients to seek employment after transplantation may improve employment rates.

Exploring parenthood in the New Zealand Heart Transplant Program.

Heart transplantation is an established treatment for end-stage cardiac disease. This study describes parenthood after heart transplantation in the New Zealand population. An analysis was performed of all heart recipients from the New Zealand program. Exclusion criteria were death within 3 months of transplantation or age <18 years at the time of the survey. Recipients (or next of kin if recipients deceased) were surveyed regarding family status at the time of transplantation and new parenthood after transplantation. A total of 145 of 199 eligible recipients completed the survey ∼12.2 years after transplant (119 male, 26 female). Before transplantation, 81% were in a permanent relationship; 72% had children. After transplantation, 19/45 recipients had 27 children (2 female recipients had 3 children), of whom 15 were planned. Complications occurred in 7/27 pregnancies (including one atrial septal defect requiring surgery). Two recipients died after the birth of their children (children aged 2.6 and 14.1 years). This study provides unique data relevant to both female and male recipients regarding new parenthood after heart transplantation and will promote more informed discussion with transplant recipients.

Oxygen therapy for pneumatosis coli: a report of two cases and a review.

Two patients with incapacitating symptoms from pneumatosis coli were successfully treated with high concentration oxygen (55-70%) inhalation via a Hudson face mask for five days. Symptoms improved dramatically starting on the third treatment day. Continuous restriction in an oxygen tent is unnecessary. This is an effective, simple and safe therapy for pneumatosis coli.

Planned pregnancy in a heart transplant recipient.

Solid organ transplantation often leads to the return of fertility in recipients. In females of childbearing age this can be accompanied by a desire to have children. The present paper discusses a planned pregnancy in a heart transplant recipient and outlines important -management issues. These principles of management can be applied to most solid organ transplant recipients.

Clinical evaluation versus Doppler echocardiography in the quantitative assessment of valvular heart disease.

We tested the hypotheses that Doppler echocardiography has a higher accuracy than clinical evaluation in the detection of significant aortic and mitral valvular heart disease and that Doppler echocardiography is highly accurate as compared with cardiac catheterization for the assessment of valvular disease severity. Thus, cardiac catheterization for the assessment of valve lesion severity may be unnecessary in selected patients. We prospectively evaluated 75 consecutive patients, ages 20-74 years (mean, 52 years), with clinically suspected valvular heart disease. Specific clinical and Doppler echocardiographic criteria were used to categorize each valve lesion as absent, insignificant, or significant. Criteria for a significant lesion at cardiac catheterization was an aortic or mitral valve area less than 1.1 or 1.5 cm2, respectively, or equal to or greater than 3+ cm2 aortic or mitral regurgitation at angiography. In all valve lesions, Doppler echocardiography had a higher overall accuracy than clinical evaluation. Increases in accuracies of 28%, 19%, 15%, and 7% occurred for mitral stenosis, aortic stenosis, aortic regurgitation, and mitral regurgitation, respectively, resulting in overall accuracies of 97%, 100%, 95%, and 96%. Clinical evaluation alone made 28 errors (37% of patients and 19% of valve lesions assessed), and 17 of these errors (23% of patients and 12% of valve lesions) would have resulted in inappropriate management. In only four (24%) of these 17 patients, the attending cardiologist would not have proceeded to assess the valve at cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary artery surgery in the elderly: long-term follow-up.

BACKGROUND: There has been controversy concerning the indications for coronary artery surgery in the elderly, particularly in countries where health resources are restricted. AIMS: To assess the results of coronary artery bypass grafting (CABG) in a large group of elderly subjects with regard to initial risks and long term follow-up. METHODS: Ninety-six consecutive patients aged 70 years or older underwent isolated CABG between January 1981 and December 1985. Long term follow-up was obtained in 94 (98%). RESULTS: The mean age was 71.6 years (70-78) and mean duration of follow-up 73 months. Seventy (73%) were male. In 80 cases the myocardial score was > 10. In 22 of 90 who had left ventricular angiography the ejection fraction was < 50%. Hospital survival was 96% and the five year survival 77%. It was not influenced by gender, myocardial score, ejection fraction or age at the time of operation. The status of survivors was reviewed in 1991. Of the 55 long term survivors 35 (64%) were free of angina. Eight (15%) and ten (18%) were in the Canadian Cardiovascular Society Angina classes 1 and 2 respectively. Seventeen patients (31%) had symptoms of heart failure with 14 (25%) in NYHA class 2 and 3 (5%) in class 3. Eight patients (15%) had survived a cerebrovascular event during follow-up. There were 35 late deaths (37%). Sixteen of these were cardiac, 18 due to other causes and one unknown.

Cytomegalovirus myocarditis in a heart transplant recipient: sensitive monitoring of viral DNA by the polymerase chain reaction.

A 54-year-old female heart transplant recipient had an acute episode of graft rejection that was treated with high-dose immunosuppression therapy. During this therapy a second febrile illness developed, which was accompanied by hypotension, anemia, and rash. Findings for subsequent myocardial biopsy specimens were negative for cytomegalovirus by culture but were strongly positive for cytomegalovirus deoxyribonucleic acid by the polymerase chain reaction. Histologic observation of viral inclusions in the myocardial fibers supported the diagnosis of cytomegalovirus myocarditis. The polymerase chain reaction therefore can provide a rapid and highly sensitive method for heart transplant patients with suspected cytomegalovirus myocarditis.