2011/09/06 by Alan R. Katritzky, Stanisław Rachwał, Anil Bhattarai · 1 citation
Biochemistry, Genetics and Molecular Biology · Chemistry · Medicine · #Congenital Heart Disease Studies #Phenothiazines and Benzothiazines Synthesis and Activities #Pulmonary Hypertension Research and Treatments #Quinazolinone synthesis and applications #Synthesis and Biological Evaluation #Vascular anomalies and interventions
paper · doi:10.1021/cr200031r
openalex publication_date 2013/01/24 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/28
SUMMARY Since the first description of the so called “one and a half ventricle repair”, published by Billingsly et al. in 1989 1, this type of correction has been applied to several complex congenital cardiac anomalies. The rationale underlying this repair is twofold. On one hand the aim is to reduce the blood flow to, and therefore to downsize the pre-load of, a dysfunctional or hypoplastic right ventricle (RV), considered to be unable to sustain a biventricular correction. On the other hand the one and a half ventricle repair (1.5 VR) permits to maintain a complete and physiological separation of pulmonary and systemic circulations, avoiding blood mixing and desaturation. Furthermore it represents a valid alternative to the Fontan circulation in the setting of a less but still functioning RV, with the advantage to provide a pulsatile pulmonary blood flow and to prevent systemic venous hypertension 2,3. One and a half ventricle repair consists of bidirectional cavo-pulmonary shunt (BCPS) in addition to complete closure of intracardiac communications, associated or not with repair of other congenital heart defects. It can be appropriate for a wide spectrum of congenital anomalies, provided that the RV is large enough to manage the blood supply from the inferior vena cava. However, feasibility in adult population is being explored 1,4. This study has divided in two parts: 1. Clinical study 2. Experimental study in animals. In clinical study we have reviewed our institutional experience with 1.5 VR to evaluate early and long term results and explore the impact of associated cardiac malformations on outcomes of the one and a half ventricle repair. Animal study in rabbits was conducted to evaluate the effect of a pulsatile venous flow pattern in superior vena cava and to understand an immediate & after pharmacologically induced stress test the hemodynamic changes in superior vena cava, right atrium, right ventricle, pulmonary artery and inferior vena cava district. A. Clinical study Methods All patients who underwent one and a half ventricle repair between March 1994 and January 2012 were included. All available clinical and operative data were reviewed. Patients were divided in: Group A - patients with right ventricle hypoplasia/dysfunction and malformations of tricuspid or pulmonary valves; Group B - patients with associated complex malformations involving other cardiac structures. Results This is an 18 years retrospective clinical review of 24 consecutive patients who underwent one and a half ventricle repair at our Institution. Mean age at repair was 11.9 years (range 4 months – 66.8 years). Mean TV annulus Z-score was -3.2 (range -6.2 to 3.6). Mean pulmonary vascular resistance was 1.75 U/m2 (range 1.0 to 3.0). Three patients had heterotaxy syndrome. Sixteen patients underwent previous cardiac operation, one of these had a Fontan operation. There was no death at operation. Postoperative complications occurred in 19 patients (79 %), and were: chylothorax/chylopericardium (n=7), BAV III (n=2), arrhythmias (n=7), renal failure (n=4), heart failure (n=4), cardiac/pleural effusion (n=11), SVC syndrome (n=2), pulmonary infection (n=3), hemi diaphragm paralysis that required diaphragm plasty (n=1), SVC thrombosis with PE (n=1). Five patients required definitive PM implantation. All patients were discharged home alive and well, after a mean hospital stay of 32 ± 29 days. At a mean follow up of 8.3 years (range 1 month – 17.9 years, FU completeness: 96 %), there were 2 late deaths (1 non cardiac related). Among survivors, functional status was NYHA class I in 19 patients (90.4%). Late adverse events occurred in 10 patients (43 %) including: late reoperation (n=3), one of these was a biventricular conversion; haemodynamic procedures (n=6); arrhythmias (n=2); neurological event (n=1); other complications (n=5). Overall freedom from adverse events, surgery and interventional procedures was 56.5 %, 87 % and 73,9 % respectively. Freedom from adverse event in Group A (n=12) was 83.3 % while in Group B (n=11) was 27.3 %. Statistical analysis demonstrated that Group B had a significantly lower freedom from adverse events than Group A (p = 0.015). B. Animal Study Methods Experiment was performed in experimental Rabbits. Animal care has taken according to established standard for experiment in animals. Experimental model: RABBIT Strain: NEW ZEALAND Total animal number: 30 Weigh of animals:4. 5-5.5 kg Results 1. After cavapulmonary anastomosis- SVC and PA pressure has increased but RA pressure has decreased, which proves the decrease of the pressure in inferior vena cava district. 2. After pharmacologically induced stress test: increased the pressure in superior vena cava and also increased heart rate but right ventricular, right atrial & pulmonary artery pressures were remained as same as after cavopolmonary anastomosis, as it is in resting condition. 3. That means superior vena cava pressure is always higher after cavapulmonary anastomosis but venous pressure in inferior vena cava district is constant and even lower in comparison with preoperative pressure. 4. We found no any changes of right atrial pressure, after 1.5 ventricular repair and even after pharmacologically induced stress test which proves that the inferior vena cava return is ok and no hypertension in inferior vena cava district even after stress test(good exercise tolerance), which is a very positive finding in favour of one and a half ventricle repair in comparison to Fontan type procedure. Conclusion After having the result of both clinical study and experiment in animals we have concluded that the 1.5 VR is a safe and valid option for surgical repair of hypoplasic, borderline or failing right ventricles as an alternative to Fontan procedure. Most patients show good functional status at follow up. This repair provides a low pressure in the inferior vena cava district and allows good early and mid-term outcomes.