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Biomedical subjects

R Lange

Publications and source records attributed to R Lange.

At least 91 records · Page 5Linked to original sources
PubMed2000

Repair of interrupted aortic arch: results after more than 20 years.

BACKGROUND: This study focused on the influence of concomitant anomalies, the individual surgical approach, and the probability for reinterventions. METHODS: Between 1975 and 1999, 94 patients with interrupted aortic arch were evaluated for short- and long-term results after surgical treatment. RESULTS: Interrupted aortic arch was associated mainly with a ventricular septal defect (85%) and left ventricular outflow tract obstruction (LVOTO, 13%). Mean follow-up was 6.7 years (median 6.9 years, 628.4 patient years). A single-stage operation was performed in 76 cases. Early mortality for two-stage procedures was 37% and late mortality was 26%, compared with single-stage procedures, with an early mortality of 12% and a late mortality of 20%, respectively. Early mortality in patients with additional LVOTO was 42% and late mortality was 50%. Freedom from reoperation at 5 years was 62%, and at 10 years was 49%. Reinterventions were performed mainly for residual arch stenosis, also with bronchus or tracheal compression, or LVOTO. CONCLUSIONS: Arch continuity and repair of associated anomalies can be achieved with an acceptable overall risk in this often complex entity. Associated anomalies play an important role in the outcome. Single-stage repair with primary anastomosis of the arch should be the surgical goal. The long-term probability for reoperation is high.

Adolescent↗
PubMed2000

An improved vector system for insertional gene inactivation inspired by the tmRNA-tagging system of S. pneumoniae.

Insertional mutagenesis is a technique often used to inactivate genes in Streptococcus pneumoniae. Using conventional vectors, a 5' segment of the targeted gene remains under the control of the gene's authentic promoter following gene disruption. Thus, the expression of a functional peptide and the misinterpretation of results in consequence cannot be excluded. To circumvent this problem, we have developed a plasmid for insertional mutagenesis based on the tmRNA-tagging system of S. pneumoniae which ensures that any protein expressed after gene disruption is degraded. Insertional mutagenesis using this vector results in the targeted gene being tagged with a tmRNA-derived sequence coding for a proteolysis tag. Here we show that the translation product of a gene tagged by this method is not detectable by Western blotting, suggesting that the protein was degraded. This modified vector allows total inactivation of genes with a reliability that cannot be achieved by conventional vectors for insertional mutagenesis. This approach can be applied to other bacterial species.

Bacterial Proteins↗
PubMed2000

Angiographic findings and clinical correlates in patients with cardiogenic shock complicating acute myocardial infarction: a report from the SHOCK Trial Registry. SHould we emergently revascularize Occluded Coronaries for cardiogenic shocK?

OBJECTIVES: We sought to delineate the angiographic findings, clinical correlates and in-hospital outcomes in patients with cardiogenic shock (CS) complicating acute myocardial infarction. BACKGROUND: Patients with CS complicating acute myocardial infarction carry a grave prognosis. Detailed angiographic findings in a large, prospectively identified cohort of patients with CS are currently lacking. METHODS: We compared the clinical characteristics, angiographic findings, and in-hospital outcomes of 717 patients selected to undergo angiography and 442 not selected, overall and by shock etiology: left or right ventricular failure versus mechanical complications. RESULTS: Patients who underwent angiography had lower baseline risk and a better hemodynamic profile than those who did not. Overall, 15.5% of the patients had significant left main lesions on angiography, and 53.4% had three-vessel disease, with higher rates of both for those with ventricular failure, compared with patients who had mechanical complications. Among patients who underwent angiography, those with ventricular failure had significantly lower in-hospital mortality than patients with mechanical complications (45.2% vs. 57.0%; p = 0.021). Importantly, for patients with ventricular failure, in-hospital mortality also correlated with disease severity: 35.0% for no or single-vessel disease versus 50.8% for three-vessel disease. Furthermore, mortality was associated with the culprit lesion location (78.6% in left main lesion, 69.7% in saphenous vein graft lesions, 42.4% in circumflex lesions, 42.3% in left anterior descending lesions, and 37.4% in right coronary artery lesions), and Thrombolysis In Myocardial Infarction (TIMI) flow grade (46.5% in TIMI 0/1, 49.4% in TIMI 2 and 26% in TIMI 3). CONCLUSIONS: Patients who underwent angiographic study in the SHOCK Trial Registry had a more benign cardiac risk profile, more favorable hemodynamic findings and lower in-hospital mortality than those for whom angiograms were not obtained. Patients with CS caused by ventricular failure had more severe atherosclerosis, and a different distribution of culprit vessel involvement but lower in-hospital mortality, than those with mechanical complications. Overall in-hospital survival correlates with the extent of coronary artery obstructions, location of culprit lesion and baseline coronary TIMI flow grade.

Aged↗
PubMed2000

Mitral-valve replacement in children under 6 years of age.

OBJECTIVE: In patients with congenital mitral-valve disease, reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, mitral-valve replacement (MVR) is the only option. We analyzed, retrospectively, data of 35 patients younger than 6 years of age, who underwent MVR at our institution. METHODS: Between 1974 and 1997, 35 children underwent MVR. The ages ranged from 2.7 months to 5.5 years (mean=1. 9+/-1.7 years) and body weight varied between 3.2 and 16.7 kg (mean=8.2+/-4 kg). The main indication (57%) for valve replacement was severe mitral-valve insufficiency. Eighteen patients (51%) had undergone at least one previous reconstructive operation (mean=1. 46+/-1.86 years) before the MVR. In 29 cases (83%), mechanical prostheses were implanted. Six patients received a bioprosthesis. The size of the prostheses ranged between 14 and 27 mm. RESULTS: The overall hospital mortality was 17.1% (6/35), and decreased from 33 (1974-1985) to 11.5% (1986-1997). Seven children died late. The actuarial survival after 20 years was 51.2+/-13.3%. Eight patients (23%) required 10 reoperations (8.2%/100 patient-years). Freedom from reoperation at 10 years was 50+/-22%. Valve-related complications were thrombo-embolism (n=2; 1.6%/100 patient-years), hemorrhage (n=1; 0.8%/100 patient-years), structural deterioration (n=3; 2.5%/100 patient-years) and non-structural dysfunction (n=3; 2. 5%/100 patient-years). Follow-up is 96% complete, with a total of 122 patient-years (mean=4.2+/-4.7 years). Eighty six percent of the patients are in New York Heart Association (NYHA) class I, 95% have sinus rhythm and 59% do not need medication. All survivors, except for those who received a bioprosthesis, were placed on a regimen of Phenprocoumon (Marcumar((R))), aiming to maintain the International Normalized Ratio (INR) between 2.5 and 3.5. In one third of these children, self-management of oral anticoagulation was performed either by the patients or their parents. CONCLUSIONS: MVR in small children still carries a high risk. In our experience, the long-term results are satisfying. After failed reconstructive surgery, or as a primary procedure, we prefer mechanical prostheses. They are well tolerated and the incidence of anticoagulation-related complications is low.

Chi-Square Distribution↗
PubMed2000

Reduction of pro-inflammatory cytokine levels and cellular adhesion in CABG procedures with separated pulmonary and systemic extracorporeal circulation without an oxygenator.

OBJECTIVE: We have recently shown that a considerable amount of pro-inflammatory cytokines is released during pulmonary passage after aortic declamping in patients undergoing coronary artery bypass grafting. The present study was performed to investigate whether bilateral extracorporeal circulation with the lungs as oxygenators can reduce the inflammatory responses of the lungs. METHODS: Eighteen consecutive patients undergoing coronary artery bypass grafting were randomly assigned to routine extracorporeal circulation with cannulation of right atrium and aorta (routine circulation, ten patients) or to a bilateral extracorporeal circulation with additional cannulation of left atrium and pulmonary artery (bilateral circulation, eight patients). Blood was simultaneously drawn from right atrium and pulmonary vein at 1, 10 and 20 min reperfusion. The levels of interleukin (IL)-6 and IL-8 and the adhesion molecules CD41 and CD62 on platelets and CD11b and CD41 on leukocytes were determined. Because of considerable interindividual scatter, the pulmonary venous levels are normalized to percent of the respective right atrial value at each time point. RESULTS: At 1 min reperfusion pulmonary venous levels of IL-6 and IL-8 in routine circulation were +44+/-15% and +43+/-28% of the respective right atrial values. The respective values in bilateral circulation were -3+/-4% and -6+/-7% (P=0.02 and P=0.05 vs. respective right atrium). Similar increments were found after 10 and 20 min. Platelet-monocyte coaggregates were retained during pulmonary passage at 1 min reperfusion in routine circulation (-21+/-6%), but washed out in bilateral circulation (+5+/-8%, P=0. 007). At 20 min reperfusion, activated polymorphonuclear neutrophils (PMN) were retained in routine circulation (-16+/-9%) but washed out in bilateral circulation (+19+/-29%, P=0.05; all data given as mean+/-SEM). CONCLUSIONS: Bilateral extracorporeal circulation without an artificial oxygenator significantly reduces the inflammatory responses during pulmonary passage after aortic declamping.

Aged↗
PubMed2000

Reconstruction of the RVOT with valved biological conduits: 25 years experience with allografts and xenografts.

OBJECTIVE: The reconstruction of the RVOT in congenital heart disease often requires the implantation of a valved conduit. Although allografts are considered the conduit of choice their availability is limited and therefore xenografts are implanted as well. We compared the long-term durability of both grafts in the RVOT over a 25-year period. METHODS: Between January 1974 and August 1999, 505 patients (median age 4.0 years, range 2 days-31 years; median weight 14.5 kg, range 2.2-76.6 kg; median body length 103 cm, range 48-183 cm) with congenital malformations (PA 25.3%, TOF 14.5%, TOF+PA 2.4%, DORV 4.2%, TGA+PS 8.7%, TAC 24.8%, and other 20.2%) received their first valved conduit (174 xenografts: median diameter 14 mm, range 8-27 mm; 331 allografts: median diameter 19 mm, range 8-30 mm). RESULTS: Follow-up is 3017 patient-years. The 10-year survival-probability for all patients. was 66% with a mean reoperation-free interval for conduit-exchange of 13.3 years (mean reoperation-free interval for allografts, 16.0 years; mean reoperation-free interval for xenograft, 10.3 years). One hundred and thirteen patients underwent a conduit-exchange, mostly due to conduit stenosis. Fourteen patients had a second exchange and three patients a third exchange. For patients with conduit diameters <18 mm (n=235: allograft n=116, xenograft n=119; median age 9 months, range 0-27.3 years), the mean reoperation-free interval was 11.2 years (mean interval allograft, 13.1 years; mean interval xenograft, 8.6 years, P=0.03). For conduit diameters >/=18 mm (n=270: allograft n=215, xenograft n=55, median age 7.4 years, range 0-34.3 years) the mean interval from freedom of conduit exchange was 15.1 years (for allografts 14.1 years, for xenografts 12.5 years, P<0.01). Comparing xenografts to allografts, we found no difference in patient survival probability (P=0.62). There was no significant difference between antibiotic (n=198) preserved vs. cryopreserved (n=133) allografts (P=0.06). Blood group compatibility of allografts to recipients had no significant influence on allograft function (P=0.42). The donors allograft origin, whether aortic or pulmonary valve, had also no significant influence on allograft long-term function (P=0.15). CONCLUSION: For the reconstruction of the right ventricular outflow tract (RVOT) allografts show significantly better long-term durability than xenografts regardless of the age at implantation and the diameter.

Adolescent↗
PubMed2000

Single centre experience on primary correction of common arterial trunk: overall survival and freedom from reoperation after more than 15 years.

OBJECTIVE: To identify predictors of early and late outcomes of common arterial trunk (CAT) after primary surgical correction, such as clinical state prior to surgery, age and weight at presentation, implications of truncal valve abnormalities and associated anomalies of this complex congenital malformation. METHODS: A consecutive series of 106 patients, operated between 1976 and 1998, were reviewed retrospectively. Using the 'Van Praagh' classification, 59 patients presented as A1, 33 as A2, six as A3, and eight as A4. The mean age at operation was 8.6 months (range, 14 days-8.9 years; median, 4.4 months), and the mean weight was 5.2 kg (range, 2.5-30.8 kg; median, 4.4 kg). At the time of operation, 32 patients had congestive heart failure, five were on ventilator support for less than 1 week, and 21 for more than 1 week. RESULTS: Between 1976 and 1989, the early mortality was 21%, and between 1990 and 1998, it dropped to 13%. After 1, 10 and 15 years, the overall survival was 66, 61 and 59%. The 1, 5, 10 and 15 year freedom from reoperation was 82, 60, 22 and 10%, respectively. Clinical condition prior to intervention, truncal valvar dysfunction, and coronary anomalies were significantly associated with poor surgical outcome, whereas weight and age at presentation in our series were not. CONCLUSIONS: In the current era of paediatric cardiac surgery, primary surgical repair of CAT can be carried out with reasonable early and late mortality. However, our data suggest that a high incidence of reoperation, mainly due to the outgrowth and failure of the conduit, has to be expected. The patient's clinical state after diagnosis is decisive for the timing of intervention.

Child↗
PubMed2000

Flexibility and stability of the structure of cytochromes P450 3A4 and BM-3.

The flexibility of the structure and compressibility of the respective active site of cytochromes P450 3A4 (CYP3A4) and BM-3 (CYP102) were studied using absorption spectroscopy in the ultraviolet and visual regions. Conformational changes in the overall protein structures of both CYP3A4 and CYP102 due to the effects of temperature and pressure are reversible. However, the enzymes differ in the properties of their active sites. The CYP3A4 enzyme denatures to the inactive P420 form relatively easy, at 3000 bar over half is converted to P420. The compressibility of its active site is lower than that of CYP102 and is greater with the substrate bound, which is in line with the observed lack of a stabilizing effect of the substrate on its conformation under pressure. In contrast, CYP102, although having the most compressible active site among the P450s, possesses a structure that does not denature easily to the inactive (P420) form under pressure. In this respect, it resembles the P450 isolated from acidothermophilic archaebacteria [McLean, M.A., Maves, S.A., Weiss, K.E., Krepich, S. & Sligar, S.G. (1998) Biochem. Biophys. Res. Commun. 252, 166-172].

Bacterial Proteins↗
PubMed2000

Vascular tracheobronchial compression syndromes-- experience in surgical treatment and literature review.

Between January 1988 and December 1997 a total of 22 patients (age: 8 days-46 years) were operated for vascular airway compression syndromes with respiratory insufficiency. Vascular anomalies in tracheal compression were double aortic arch in 7 patients, (2 previously operated elsewhere), right aortic arch + left ligamentum arteriosum in 1, and pulmonary artery sling in 3. Three of these patients had secondary long-segment tracheomalacia. Compression of trachea and a main bronchus existed in 2 patients with right aortic arch + left ligamentum. Isolated main bronchus obstruction was present in 9 patients (abnormal insertion of ligamentum arteriosum in 1, status post (s.p.) previous operation for PDA in 4, s. p. surgery for coarctation in 1, right aortic arch + left ligamentum arteriosum in 2, and right lung aplasia + left ligamentum in 1). 3 of these cases had secondary long-segment bronchomalacia. All patients had a complex respiratory anamnesis [long-term intubation in 7, s.p. tracheostomy in 2 (over 3 months - 3 years), and progressive respiratory insufficiency in 13). In tracheal compression, surgical correction included transsection of the underlying ring or sling components (with additional anterior aortic arch translocation in 5 patients resection-reimplantation of left pulmonary artery in 3, segmental tracheal resection in 1, and external tracheal suspension in 2). In the 2 cases with compression of the trachea and a main bronchus, aortic "extension" by a prosthetic tube was necessary. In isolated main bronchus obstruction, surgical decompression basically consisted of transsection of the ligamentum arteriosum or resection of its scarry remnant forming the "corner point" of a compression between aorta and pulmonary artery. In 3 patients with secondary long-segment malacia, additional external bronchus suspension was performed. Effective decompression and re-expansion of the airway segment concerned was achieved, and was demonstrated by intraoperative endoscopy in all patients. There were 3 postoperative deaths (sepsis 2; massive, irreversible edema of the tracheal mucosa 1). Of the 19 surviving patients 16 could be extubated between the 1st and 17th (mean = 7.5) postoperative day. In 1 case the preoperative long-term tracheostomy had to be left in place for inoperable additional laryngeal stricture. 2 patients had to be reoperated (segmental cervical tracheal resection after 5 months for primary long-term intubation-related subglottic stenosis in 1, esophageal decompression for residual dysphagia after 57 months related to a traction phenomenon at the right descending aorta in the other), both with gratifying results. In all other patients clinical, endoscopic, and radiographic examinations (follow-up = 2 months - 6 years) demonstrate good results.

Adolescent↗
PubMed2000

Effect of cardiopulmonary bypass and hemofiltration on plasma cytokines and protein leakage in pigs.

BACKGROUND: The hypothesis that an inflammatory process during and after cardiopulmonary bypass (CPB) impairs hemodynamics and causes increased capillary protein leakage and that this is possibly ameliorated by hemofiltration (HF) was tested. METHOD: 26 anesthetized pigs were subjected to 120 min CPB (90 min cardioplegia followed by 30 min reperfusion, combined with conventional and modified HF in 13 animals). Hemodynamics, leukocytes, cytokines (IL-1ra, IL-8, IL-10, TNF-alpha), LNPI, plasma protein, and the half-life of i.v. injected Evans Blue (t/2) were assessed before and after CPB. RESULTS: CPB was followed by depression of left ventricular function and activation of inflammatory mediators. Although a slight elimination of some inflammatory mediators occurred, HF did neither improve cardiac function nor reduce the inflammatory process. Plasma protein was lost during CPB and hemofiltration by protein trapping to the surfaces of the CPB system, by filtration across the hemofilter, and by increased microvascular filtration (solvent drag). The latter was probably due to an increased filtration pressure in consequence of the reduction of plasma colloid osmotic pressure by the crystalloid primed CPB. t/2 did not indicate an increased microvascular protein leakage after CPB. CONCLUSION: Hemofiltration is ineffective in improving cardiac function or reducing the inflammatory response of CPB in the pig model.

Animals↗
PubMed2000

Acetylsalicylic acid effervescent 1000 mg (Aspirin) in acute migraine attacks; a multicentre, randomized, double-blind, single-dose, placebo-controlled parallel group study.

In this multicentre, randomized, double-blind, single-dose study a total of 374 patients generally suffering from migraine attacks suitable for treatment with non-prescription drugs, received either oral acetylsalicylic acid effervescent 1000 mg (ASAE) or effervescent placebo for the treatment of an acute migraine attack. Of the 343 patients fulfilling the criteria for efficacy analysis 169 patients took acetylsalicylic acid and 174 placebo. Response rates (reduction of headache severity from severe or moderate to mild or no pain at 2 h after administration) were 55.0% for acetylsalicylic acid and 36.8% for placebo (P < 0.001). Twenty-nine percent of patients in the active treatment group were pain-free after 2 h compared with 16.7% in the placebo group (P = 0.007). No headache recurred within 24 h post-dose in 84.6% of patients in the active group and in 85.1% of patients in the placebo group. Effervescent placebo reduced nausea and vomiting to the same degree as the active drug. Adverse events of acetylsalicylic acid (8.3%) were generally mild or moderate and comparable to those of placebo (2.9%). This study shows that oral ASAE is safe and effective for the treatment of acute migraine attacks.

Acute Disease↗
PubMed2000

Microarray-based identification of a novel Streptococcus pneumoniae regulon controlled by an autoinduced peptide.

We have identified in the Streptococcus pneumoniae genome sequence a two-component system (TCS13, Blp [bacteriocin-like peptide]) which is closely related to quorum-sensing systems regulating cell density-dependent phenotypes such as the development of genetic competence or the production of antimicrobial peptides in lactic acid bacteria. In this study we present evidence that TCS13 is a peptide-sensing system that controls a regulon including genes encoding Blps. Downstream of the Blp TCS (BlpH R) we identified open reading frames (blpAB) that have the potential to encode an ABC transporter that is homologous to the ComA/B export system for the competence-stimulating peptide ComC. The putative translation product of blpC, a small gene located downstream of blpAB, has a leader peptide with a Gly-Gly motif. This leader peptide is typical of precursors processed by this family of transporters. Microarray-based expression profiling showed that a synthetic oligopeptide corresponding to the processed form of BlpC (BlpC*) induces a distinct set of 16 genes. The changes in the expression profile elicited by synthetic BlpC* depend on BlpH since insertional inactivation of its corresponding gene abolishes differential gene induction. Comparison of the promoter regions of the blp genes disclosed a conserved sequence element formed by two imperfect direct repeats upstream of extended -10 promoter elements. We propose that BlpH is the sensor for BlpC* and the conserved sequence element is a recognition sequence for the BlpR response regulator.

Alleles↗
PubMed1999

Spectral properties of the oxyferrous complex of the heme domain of cytochrome P450 BM-3 (CYP102).

Here we describe for the first time the formation of a complex of reduced CYP102 (cytochrome P450 BM-3) heme domain with molecular oxygen. To stabilize the oxycomplex, the experiments had to be done under argon atmosphere at cryogenic temperatures (-25 degrees C) in the presence of 50% glycerol. The spectral properties of this species were different from those of another P450-type autosuffisant enzyme, i.e., the neuronal nitric oxide synthase. On the contrary, the oxyferrous complex of CYP102 possesses spectral properties similar to those of complexes of microsomal cytochromes P450, e.g., CYP2B4.

Animals↗
PubMed1999

Pressure versus heat-induced unfolding of ribonuclease A: the case of hydrophobic interactions within a chain-folding initiation site.

To investigate the characteristics of the postulated carboxy terminal chain-folding initiation site in bovine pancreatic ribonuclease A (RNase A) (residues 106-118), important in the early stages of the folding pathway, we have engineered by site-directed mutagenesis a set of 14 predominantly conservative hydrophobic variants of the protein. The stability of each variant has been compared by pressure and temperature-induced unfolding, monitored by fourth derivative UV absorbance spectroscopy. Apparently simple two-state, reversible unfolding transitions are observed, suggesting that the disruption of tertiary structure of each protein at high pressure or temperature is strongly cooperative. Within the limits of the technique, we are unable to detect significant differences between the two processes of denaturation. Both steady-state kinetic parameters for the enzyme reaction and UV CD spectra of each RNase A variant indicate that truncation of hydrophobic side chains in this region has, in general, little or no effect on the native structure and function of the enzyme. Furthermore, the decreases in free energy of unfolding upon pressure and thermal denaturation of all the variants, particularly those modified at residues 106 and 108, suggest that the hydrophobic residues and side chain packing interactions of this region play an important role in maintaining the conformational stability of RNase A. We also demonstrate the potential of Tyr115 replacement by Trp as a non-destabilizing fluorescence probe of conformational changes local to the region.

Animals↗
PubMed1999

Fluorescence and FTIR study of the pressure-induced denaturation of bovine pancreas trypsin.

The pressure denaturation of trypsin from bovine pancreas was investigated by fluorescence spectroscopy in the pressure range 0. 1-700 MPa and by FTIR spectroscopy up to 1000 MPa. The tryptophan fluorescence measurements indicated that at pH 3.0 and 0 degrees C the pressure denaturation of trypsin is reversible but with a large hysteresis in the renaturation profile. The standard volume changes upon denaturation and renaturation are -78 mL.mol-1 and +73 mL.mol-1, respectively. However, the free energy calculated from the data in the compression and decompression directions are quite different in absolute values with + 36.6 kJ.mol-1 for the denaturation and -5 kJ. mol-1 for the renaturation. For the pressure denaturation at pH 7.3 the tryptophan fluorescence measurement and enzymatic activity assays indicated that the pressure denaturation of trypsin is irreversible. Interestingly, the study on 8-anilinonaphthalene-1-sulfonate (ANS) binding to trypsin under pressure leads to the opposite conclusion that the denaturation is reversible. FTIR spectroscopy was used to follow the changes in secondary structure. The pressure stability data found by fluorescence measurements are confirmed but the denaturation was irreversible at low and high pH in the FTIR investigation. These findings confirm that the trypsin molecule has two domains: one is related to the enzyme active site and the tryptophan residues; the other is related to the ANS binding. This is in agreement with the study on urea unfolding of trypsin and the knowledge of the molecular structure of trypsin.

Anilino Naphthalenesulfonates↗
PubMed1999

[Hepatopulmonary syndrome].

ANAMNESIS: We report about a 60-year-old woman with hepatopulmonary syndrome. The patient suffered from progressive dyspnea and liver cirrhosis following viral hepatitis C, acquired through blood transfusion 30 years ago. EXAMINATION RESULTS: Remarkable were clinical signs of chronic liver disease (cutaneous spider naevi, palmar erythema) and signs of chronic respiratory failure (clubbing) with platypnea. Arterial pO2 was 33 mm Hg under 41 O2/min nasal oxygen supplement and improved to 74 mm Hg under 81 O2/min. Arterial pO2 deteriorated in upright position (orthodeoxia). Contrast enhanced two-dimensional transthoracic echocardiography showed a delayed microbubble opacification in the left heart chambers (without atrial or ventricular septal defect), suggesting pulmonary vascular dilatations. Perfusion scan with macroaggregated albumin labelled with technetium-99m showed uptake over thyroid, kidneys, liver, and spleen suggesting an abnormal passage through the pulmonary vascular bed. The radiological examination with chest radiography and high resolution computer tomography revealed no signs of interstitial lung disease. The pulmonary artery angiography showed neither anatomical shunts nor malformations. DIAGNOSIS: The final diagnosis was hepatopulmonary syndrome Type 1. TREATMENT/FOLLOW-UP: The patient underwent liver transplantation with following good function of the liver graft. After 6 months the patient presented in a much better general condition, but with persisting severe hypoxemia whereas the spider naevi disappeared completely. One year after the liver transplantation in the perfusion scan the uptake over thyroid, kidneys, liver, and spleen was absent. The orthodeoxia and hypoxemia improved evidently. From that we conclude that the pulmonary vascular dilatations diminished after liver transplantation.

Diagnosis, Differential↗